Caroli’s disease is a rare congenital disorder of the intrahepatic biliary tree. A localized form of this disorder affects mainly the left lobe of the liver and can occur in early childhood. Caroli’s syndrome consists of Caroli’s disease and congenital hepatic fibrosis and as a standalone disorder is being diagnosed more often. This article reports a case of a 49-year-old woman with localized form of Caroli’s disease. The malformation affected the right lobe of the liver and was complicated by liver abscesses. Therapeutic options for this disorder rely on the clinical features and the location of the abnormality. Treatment consists of a conservative, endoscopic or surgical approach. Orthotopic liver transplantation is the ultimate management option in the most advanced forms of Caroli’s disease. Our patient underwent right hemi-hepatectomy. There were no complications during the postoperative period. She was discharged from hospital on the 7th day after resection.