Biliary cystadenocarcinoma is a rare tumor of the intrahepatic biliary trac
t, which frequently develops in a preexisting benign biliary cystadenoma. I
n the present case, diagnosis was difficult because of the lack of specific
ity of clinical, biological and radiological findings. The correct diagnosi
s was only achieved by histological examination of the resected lesion. Mac
roscopically, the right lobe of the liver showed evidence of a whitish, mul
tilolobed, malignant mass of about 6 cm in diameter Upon light microscopic
analysis, cysts were Sound to be lined with papillary forms. In some areas,
epithelial cells were clearly malignant contrasting with persistent non dy
splasic areas, suggesting the presence of underlying cystadenomas. Eleven m
onths after complete surgical resection, the patient is in good condition w
ith no evidence of recurrence.