Conselho Nacional de Desenvolvimento Cientı´fico e Tecnolo´gico (CNPq). B.B-D received a research fellowship from the Coordenac¸ão de Aperfeic¸oamento de Pessoal de Nı´vel Superior (CAPES, finance code: 001). M.B.A received a fellowship from the Fundac¸ão de Amparo à Pesquisa da Bahia (FAPESB). MAP received a fellowship from the Coordenac¸ão de Aperfeic¸oamento de Pessoal de Nı´vel Superior (CAPES). J.M.C-A was supported by the Organization of American States - Partner-ships Program for Education and Training (OAS-PAEC) and his study was financed in part by the Coordenac¸ão de Aperfeic¸oamento de Pessoal de Nı´vel Superior - Brazil (CAPES) - Finance Code 001.Neurofibromatosis type 1 (NF-1) is an autosomal dominant disease that affects one in every 3000 individuals. This disease can present a wide range of clinical manifestations, ranging from skin abnormalities to severe vascular damage. Although not commonly recognized in the context of NF-1, cerebrovascular disease (CVD), can be often present since childhood and diagnosed just later in life. When present, NF-1-associated CVD clinical manifestations may include headache, cognitive deficits and ultimately aneurysm rupture, causing death. Thus, CVD plays an important role in the clinical manifestations, disease severity and prognosis of patients with NF-1. This systematic review aims to summarize the body of evidence linking NF-1 and CVD in children. Methods Two independent investigators performed a systematic review on the PubMed and EMBASE search platforms, using the following key terms: “neurofibromatosis type 1”, “Von Recklinghausen’s disease”, "children", "adolescents", "stroke", "Moyamoya disease", "vascular diseases", "cerebrovascular disorders", "aneurysm" and "congenital abnormalities". Studies focused on assessing the development of CVD in children with NF-1 were included. Results Seven studies met the inclusion criteria. Twelve different clinical manifestations have been associated with cerebrovascular changes in children with NF-1; 44,5% of diagnosed patients were asymptomatic. The available evidence suggests that CVDs are related with the progression of NF-1, even in the absence of a clear clinical manifestation. In addition, improved prognosis was observed when imaging tests were performed to screen for cerebrovascular alterations early during the clinical investigation. Early diagnosis of CVD in NF-1 patients foster implementation of timely interventions, directly impacting clinical outcomes.