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Caroli disease (CD) and Caroli syndrome are rare genetic liver disease, characterized by intra-hepatic or may be extra-hepatic bile duct dilatation. The Caroli disease can be widely dispersed or confined and can arise in a sac that creates cystic structures that interfere with the biliary tree.Caroli disease occurs irregularly without any family history. While rare cases with auto omal dominant ancestry and involvement with autosomal dominant polycystic kidney disease have been identified. Caroli disease are characterized by symptoms such as yellowing of skin, pain etc. Caroli disease and caroli syndr me having similar symptoms, in which caroli syndrome are characterized with episodes of itching and abdomen p in. Signs of caroli disease or syndrome are more frequent in female than mal. Caroli disease and Caroli syndrome are due to genetic disorder conditions. In caroli disease, dilation of bile duct leads to cholangitis, the treatment of caroli disease includes use of antibiotics, hepatectomy, and internal bypass surgery.