Melanotic schwannoma (MS) is an unusual variant of nerve sheath neoplasm. Only 10% of these tumors will undergo malignant degeneration, with exceedingly rare reported metastasis. We present a 32-yearold woman with a 6-month history of cervical pain and left arm progressive weakness. Neurological examination showed a left upper limb radicular pain, with pyramidal syndrome at C5 level. The magnetic resonance imaging (MRI) study highlighted an intradural extramedullary heterogeneous mass along the spinal cord at the C4-C5 level, slightly hyperintense with T1 and hypointense with T2-weighted sequences, invading the left neural foramen. The patient underwent C3-C5 laminectomy with total resection of a black tumor. In the postoperative period, a patent deficit of shoulder abduction ensued related to the nervous section. Microscopically, compactly fascicles of spindle-shaped cells with pleomorphic and hypercromatic nuclei, dark brown intracellular pigments, as well as some mitotic figures were seen. Immunohistochemical stains for S-100, Human Melanoma Black-45 (HMB-45), and vimentin were positive, with Ki-67 labelling index (LI) of 15% compatible with MS. Six months after radiotherapy she presents local recurrence and lung metastatic dissemination of the MS. She underwent left pulmonary segmentectomy, followed by chemotherapy and radiosurgery. The patient developed a febrile neutropenia and worsening of general status, and died after 3 months due to respiratory complications. MS are rare tumors with potential for local recurrence and distal metastasis. Complete surgical resection remains as the treatment of choice, once the uncommon cases with malignant progression shows low response to chemo and radiotherapy.5310712716Ditz, C., Brunswig, K., Meyer, C., Reusche, E., Nowak, G., Tronnier, V., Intracranial melanotic schwannoma: A case report of recurrence with extra-and intradural manifestation two decades after initial diagnosis and treatment (2011) Cent Eur Neurosurg, 72, pp. 211-214El Benna, N., Ouachtou, K., Mahroug, N., Abdelouafi, A., Karkouri, M., Melanocytic cervical schwannoma (2009) J Neuroradiol, 36, pp. 114-115. , (French)Er, U., Kazanci, A., Eyriparmak, T., Yigitkanli, K., Senveli, E., Melanotic schwannoma (2007) J Clin Neurosci, 14, pp. 676-678Hodson, J.J., An intra-osseous tumour combination of biological importance-invasion of a melanotic schwannoma by an adamantinoma (1961) J Pathol Bacteriol, 82, pp. 257-266Hoover, J.M., Bledsoe, J.M., Giannini, C., Krauss, W.E., Intramedullary melanotic schwannoma (2012) Rare Tumors, 4, pp. e3Horvath, A., Bossis, I., Giatzakis, C., Levine, E., Weinberg, F., Meoli, E., Robinson-White, A., Stratakis, C.A., Large deletions of the PRKAR1A gene in Carney complex (2008) Clin Cancer Res, 14, pp. 388-395Huang, H.Y., Park, N., Erlandson, R.A., Antonescu, C.R., Immunohistochemical and ultrastructural comparative study of external lamina structure in 31 cases of cellular, classical, and melanotic schwannomas (2004) Appl Immunohistochem Mol Morphol, 12, pp. 50-58Kaehler, K.C., Russo, P.A., Katenkamp, D., Kreusch, T., Neuber, K., Schwarz, T., Hauschild, A., Melanocytic schwannoma of the cutaneous and subcutaneous tissues: Three cases and a review of the literature (2008) Melanoma Res, 18, pp. 438-442Killeen, R.M., Davy, C.L., Bauserman, S.C., Melanocytic schwannoma (1988) Cancer, 62, pp. 174-183Küsters-Vandevelde, H.V., van Engen-van Grunsven, I.A., Küsters, B., van Dijk, M.R., Groenen, P.J., Wesseling, P., Blokx, W.A., Improved discrimination of melanotic schwannoma from melanocytic lesions by combined morphological and GNAQ mutational analysis (2010) Acta Neuropathol, 120, pp. 755-764Marton, E., Feletti, A., Orvieto, E., Longatti, P., Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature (2007) J Neurosurg Spine, 6, pp. 591-599Peltier, J., Page, C., Toussaint, P., Bruniau, A., Desenclos, C., Le Gars, D., Melanocytic schwannomas. Report of three cases (2005) Neurochirurgie, 51, pp. 183-189Rodriguez, F.J., Folpe, A.L., Giannini, C., Perry, A., Pathology of peripheral nerve sheath tumors: Diagnostic overview and update on selected diagnostic problems (2012) Acta Neuropathol, 123, pp. 295-319Sauka-Spengler, T., Bronner-Fraser, M., A gene regulatory network orchestrates neural crest formation (2008) Nat Rev Mol Cell Biol, 9, pp. 557-568Shields, L.B., Glassman, S.D., Raque, G.H., Shields, C.B., Malignant psammomatous melanotic schwannoma of the spine: A component of Carney complex (2011) Surg Neurol Int, 2, p. 136Smith, A.B., Rushing, E.J., Smirniotopoulos, J.G., Pigmented lesions of the central nervous system: Radiologic-pathologic correlation (2009) Radiographics, 29, pp. 1503-1524Vallat-Decouvelaere, A.V., Wassef, M., Lot, G., Catala, M., Moussalam, M., Caruel, N., Mikol, J., Spinal melanotic schwannoma: A tumour with poor prognosis (1999) Histopathology, 35, pp. 558-566Vezzosi, D., Vignaux, O., Dupin, N., Bertherat, J., Carney complex: Clinical and genetic 2010 update (2010) Ann Endocrinol, 71, pp. 486-493Welling, L.C., Guirado, V.M., Tessari, M., Felix, A.R., Zanellato, C., Figueiredo, E.G., Taricco, M.A., Teixeira, M.J., Spinal melanotic schwannomas (2012) Arq Neuropsiquiatr, 70, pp. 156-157Yeh, I., Argenyi, Z., Vemula, S.S., Furmanczyk, P.S., Bouffard, D., McCalmont, T.H., Plexiform melanocytic schwannoma: A mimic of melanoma (2012) J Cutan Pathol, 39, pp. 521-525Zhang, H.Y., Yang, G.H., Chen, H.J., Wei, B., Ke, Q., Guo, H., Ye, L., Zhang, Y.H., Clinicopathological, immunohistochemical, and ultrastructural study of 13 cases of melanotic schwannoma (2005) Chin Med J, 118, pp. 1451-1461