Isolated Caroli's disease presenting as an exophytic mass in the liver

A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identified. At laparotomy, the mass was found at the inferior border of the right lobe of the liver next to the gallbladder and was easily removed by wedge excision. Intraoperative liver biopsies and histologic findings of the excised lesion confirmed the diagnosis.