Caroli disease is a congenital disorder characterized by multifocal, segmental dilatation or ectasia of large intrahepatic bile ducts. The disease develops due to a remodeling defect, but its molecular pathogenesis is not fully understood. The absence of specific symptoms and signs in Caroli's disease complicates the diagnosis. The magnetic resonance cholangiography (MRCP) is the most sensitive method in diagnosis. Prognosis depends on the degree of liver fibrosis and liver dysfunction and whether or not renal dysfunction is present. In this article, we present a 1.5-year-old female patient diagnosed with Caroli's disease at the age of 8 months. Key Words: Caroli disease, cystic dilation, children Special Issue of Health Sciences DOI: 10.7176/JSTR/6-03-03