RÉSUMÉ
Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. We present the case of a 28-year-old woman who was incidentally diagnosed with type II Abernethy malformation and multiple aneurysms during an investigation for nonspecific abdominal pain and fever. The patient had been diagnosed with Caroli's disease at the age of 10 and liver cirrhosis, portal hypertension a few years before. To the best of our knowledge, this is the first case reported with all such congenital anomalies associated together. Ultrasound, computed tomography, including three-dimensional reconstruction, and magnetic resonance imaging were performed which revealed a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava, multiple aneurismal cystic dilation of the spleen artery and left renal artery, and extensive intrahepatic bile duct cysitic dilation with calculus formation. Etiology, clinical significance and management strategies with regard to these abnormalities are discussed.