OBJECTIVE A clinical case report and literature review of the on the biliary-duct hamartoma is presented. BACKGROUND The biliary hamartoma is a rare hepatic lesion described for the first time in 1918 by Von Meyenburg. It is consistent with biliary duct deformity and disorganization, forming cystic structures varying in sizes. METHODS The patient is a 44-year-old female who had an ultrasonogram that showing disseminated hepatic lesions, which rendered an irregular shape to the viscera. An abdominal CT scan corroborated this hepatic lesion that appeared as multiple and low density in cystic form, disseminated through the entire organ. This study also showed a pelvic lesion in the left ligament of the uterus. The images were interpreted as malignant left ovaric tumor with hepatic metastases. The patient was taken to exploratory laparotomy. Liver biopsies and radical hysterectomy were performed. RESULTS The frozen sections were reported as negative for malignancy and definitive diagnostic was biliary duct hamartoma and uterine myomatosis. CONCLUSIONS The presence of biliary duct hamartoma causes diagnostic confusion and difficulty in the management of patients in that it appears to be liver metastases.