Four patients, one woman and three men, with biliary cystadenocarcinoma were surgically treated during a 7-year period. The mean age was 58 years. Symptoms prior to admission were mild, and blood tests including liver function tests, CEA and AFP were for the most part within the normal range. Surgery followed diagnostic work-up including US, ERCP, CT, angiography and MRI. Radical surgery was accomplished in three patients by extended left lobectomy in two and extended right lobectomy in one patient, respectively, in this latter patient preceded by therapeutic embolization of the right portal branch prior to resection, while the fourth patient had a palliative resection. Histological examination revealed papillary adenocarcinoma with mucin production within the cyst. The size of the cyst (maximum diameter) varied between 4.5 and 24 (mean 12) cm. The postoperative course was uneventful in all patients. Two patients are alive with no sign of recurrence 5 and 53 months after surgery, while two patients died after 26 and 75 months, respectively. Thus, radical resection of this rare kind of malignant hepatic tumor seems to offer a chance of long-time survival.