Background:
Abernethy malformation is a rare splanchnic vascular abnormality characterizing extrahepatic abnormal shunts that is classified into types I and II. Abernethy malformation type I has a female predilection and is associated with a variety of concurrent hepatic benign or malignant tumours while type II with concurrent tumours is very rare in females.
Case report:
We report a rare female case of Abernethy malformation type II with concurrent occupying lesion in the right liver, which was successfully transplanted; the occupying lesion was pathologically proven to be nodular hyperplasia.
Conclusion:
This case might provide further knowledge regarding Abernethy malformation. On imaging, the anatomy of portal vein should be carefully investigated to categorize Abernethy malformation, and a wide variety of differential diagnosis of concurrent occupying lesions should be taken into account.