Abstract: Congenital absence of portal vein is a kind of abnormal congenital embryonic development which is rarely seen in clinical practice. It is easily misdiagnosed due to the lack of characteristic clinical manifestations. In this article, the computed tomography findings of 1 patient with congenital absence of portal vein associated with remodeling of the liver were analyzed. The patient was admitted to the First Hospital of Ningbo in February 2013. Relevant literatures were reviewed to investigate the diagnostic value of imaging examination, and to improve the awareness of surgeons on this diseases.