Congenital intrahepatic bile duct dilatation (Caroli's disease) is a rare biliary disease. Although multiple reports exist describing its surgical treatment, relatively few have provided long-term follow-up. Prospective data about 25 cases of monolobular Caroli's disease, with liver resection between 1974 and 2016, were retrospectively analyzed. Patient demographics together with postoperative outcomes and long-term follow-up were assessed. Our 25-patient cohort (average age 53.4 years (range: 27-82)) included 20 cases with disease limited to the left lobe, and 5 to the right. The average time interval between first symptoms and final diagnosis was 5 years (range: 0-34 years). The surgical procedures included left lobectomy in 11 cases, left hepatectomy in 8 cases, right hepatectomy in 3, and sub-segmentectomy in 3 cases. Biliodigestive anastomosis was performed in 7 cases. Complications were observed in 3 patients (25%). Metachronous cholangiocarcinoma was observed in one single case, 10 years after initial operation. In conclusion, surgical treatment for monolobular Caroli's disease is effective, with good short-term results and few complications. Median long-term follow-up was 18 months (range: 3-132), with favorable clinical evolution in 96% of patients.