Introduction: Bile duct hamartomas (BDH) are benign liver lesions that have been attributed to malformations of the bile duct plate, leading to focal areas of hepatic tissue containing multiple, malformed and dilated bile ducts which are set in the background of fibrous stroma. BDH are considered a spectrum of fibropolycystic liver disease, which includes congenital hepatic fibrosis, autosomal dominant polycystic disease, and Caroli disease. Methods: We reviewed all patients who had an MRI suggestive of BDH located in the last 2 years (2007-2008); for inclusion in the study, patients needed confirmatory histologic evidence of this diagnosis. Every patient was offered an operation based on symptoms; unresected patients were excluded. Patients with florid Polycystic disease of the liver were also excluded. Whenever feasible, the laparoscopic approach (fenestration) was preferred; however, segmental resections were done to avoid recurrence of the BDH. Follow-up was achieved through clinic outpatient visit.Results: Twenty patients met the inclusion criteria (M:F 15:5). Average age was 61.3 yr (SD 12.5). Symptoms prompting the operation were abdominal pain (n=18), jaundice (n=1), hepatic abscess (n=1); the last 2 complications were related to previous interventional manipulation of the cyst. 7 patients had history and histologic evidence of previous bleeding (presently or immediately past). 8 patients received a laparoscopic approach, 4 were started laparoscopically and converted to open (3 for hemostasis, 1 for bile duct exploration) and 8 received an open approach due to the complexity of the cyst, its location or the complication related to it (jaundice, infection). LOS was 2.4 d (SD 1.8) for the laparoscopic approach, 4 d (SD 1) for the conversion and 9.2 d (SD 4.6) for the open procedures. Complications included wound infection, biloma, intraoperative hypotension, renal failure, abdominal abscess (1 each one), all patients with open approaches. Of 18 patients available for follow-up (the other 2 have less than 4 wk FUP), all patients resolved the symptom that prompted the operation. Postoperative imaging ruled out recurrence of the BDH.Conclusions: BDH are benign conditions occurring in the liver that respond very well to operative treatment. Whenever possible, laparoscopic fenestration and/or resection are the preferable choice. Intensive interventional manipulation of the cyst results in complications, both for the approach and postoperative period.