Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman

We present a new case of congenital absence of the portal vein and focal nodular hyperplasia in the liver without additional congenital anomalies. Ultrasound, computed tomography, magnetic resonance imaging, and angiography depicted the splenic vein and the superior mesenteric vein joining and entering into the inferior vena cava without passing through the liver. The features of this patient and the 30 previously reported cases are reviewed.