Caroli's disease is characterized by congenital non-obstructive dilatation of the intrahepatic bile ducts of undefined etiology. It is a rare disease usually affecting the whole liver but it may affect a lobe or a segment (11). This study shows the evolution of 6 cases (2 boys and 4 girls) that were diagnosed with Caroli's disease at a referral service. Their ages ranged from 2 to 16 years - median age 10 years. One of the patients presented with cholangitis, while hepatomegaly was observed in 83% of the cases. Four of the patients presented biliary lithiasis and in one of these cholesterol crystals could be observed in the duodenal secretion. The diagnosis was confirmed in 4 cases by endoscopic retrograde cholangiopancreatography, in 1 by cholangioresonance and 1 by echography. Two of the patients also presented congenital hepatic fibrosis. The outpatient clinic follow-up indicated that all the patients evolved well with the exception of one patient who was selected as a candidate for liver transplantation.3314751Asselah, T., Ernst, O., Sergent, G., Caroli's disease: A magnetic resonance cholangiopancreatography diagnosis (1988) Am J Gastroenterol, 93 (1), pp. 109-110Borges Pinto, R., Lima, J.P., Silveira, T.R., Caroli's Disease: Report of 10 cases in children and adolescents in Southern Brazil (1998) J Pediatr Surg, 33 (10), pp. 1531-1535Cabrera, J., Quintero, E., Bruguera, M., Diagnosis of Caroli's disease by technetium-99m DISIDA cholescintigraphy. Report of three cases (1985) Clin Nucl Med, 10 (7), pp. 478-482Caroli, J., Corcos, V., Maladies des voies biliaires intrahepatiques segmentales (1958) Masson Cie., Paris, 34, pp. 488-495Choi, B.I., Yeon, M.K., Kim, S.H., Caroli disease: Central dot sign in CT (1990) Radiology, 174, pp. 161-163D'Ágata, I.D.A., Jonas, M.M., Perez-Atayde, Combined cystic disease of the liver and kidney (1995) Semin Liver Dis, 14, pp. 215-228Desmet, V.J., Congenital diseases of intrahepatic bile ducts: Variations on the theme "Ductal plate malformation" (1992) Hepatology, 16 (4), pp. 1069-1081Desroches, J., Spahr, L., Leduc, F., Non-invasive diagnosis of Caroli's syndrome associated with congenital hepatic fibrosis using hepatobiliary scintigraphy (1995) Clin Nucl Med, 20, pp. 512-514Guntz, P., Coppo, G.B., Lorimier, G., La maladie de Caroli unilobaire. Aspects anatomocliniques. Démarche diagnostique et thérapeutique. A propos de 3 observations personnelles et de 101 observations de la littérature (1991) J Chir, 28, pp. 167-181Keane, F., Hadzic, N., Wilkinson, M.L., Neonatal presentation of Caroli's disease (1997) Arch Dis Child Fetal Neonatal, 77, pp. 145-146Landen, S., Bardaxoglou, E., Maddern, G.J., Caroli's disease: A surgical dilemma (1993) Acta Chir Belg, 93, pp. 224-226Marchal, G.J., Desmet, V.J., Proesmans, W.C., Caroli disease: High-frequency US and pathologic findings (1986) Radiology, 158, pp. 507-511Nari, G., Prenna, J., Moreno, E., Enfermidad de Caroli: Presentation de un caso y revision de la literatura (1998) Acta Gastroent Latinoameri, 28 (2), pp. 209-211Oria, A., Enfermidad de Caroli: Un dilema terapeutico en una patologia mal definida (1998) Acta Gastroent Latinoameri, 28 (2), p. 227Pavone, P., Laghi, A., Catalano, C., Caroli's disease: Evaluation with MR cholangiopancreatography (MRCP) (1996) Abdom Imaging, 21, pp. 117-119Ros, E., Navarro, S., Bru, C., Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli's syndrome (1993) Lancet, 342, pp. 404-406Ruffa, G., Lucigrai, G., Bartocci, M., Aspetti ecografici della malatia di Caroli (1996) Minerva Pediatr, 48 (7-8), pp. 337-340Sans, M., Rimola, A., Navasa, M., Liver transplantation in patients with Caroli's disease and recurrent cholangitis (1997) Transpl Int, 10 (3), pp. 241-244Schiano, T.D., Fiel, M.I., Miller, C.M., Adult presentation of Caroli's syndrome treated with orthotopic liver transplantation (1997) AJG, 92 (10), pp. 1938-1940Sood, G.K., Mahapatra, J.R., Khurana, A., Caroli disease: Computed tomography diagnosis (1991) Gastrointest Radiol, 16, pp. 243-244Tomà, P., Lucigrai, G., Pelizza, A., Sonographic patterns of Caroli's disease: Report of 5 new cases (1991) J Clin Ultrasound, 19, pp. 155-161Tritapepe, R., Treatment of intrahepatic calculi in Caroli's disease by non-invasive saline washout (1996) Panminerva Med, 38 (1), pp. 48-50Tsuchida, Y., Sato, T., Sanjo, K., Evaluation of long-term results of Caroli's disease: 21 Years' observation of a family with autosomal "dominant" inheritance and review of the literature (1995) Hepato-Gastroenterol, 42, pp. 175-181Vázquez-Iglesias, J.L., Garcia-Reinoso, C., Arnal, F., Enfermidad de Caroli: Aportación de ocho casos estudiados por CPRE (1991) Rev Esp Enferm Dig, 80, pp. 35-40