We had the opportunity to dissect an autopsy case who had developed a rare portal collateral pathway due to increased portal pressure resulting from liver cirrhosis and simultaneous abnormal left gastric venous distribution. The portal collateral pathway consisted of a well-developed communicating branch located between the left renal vein and the left gastric vein. The left gastric vein did not merge into the portal vein, but directly entered the liver after bifurcating near the hepatic hilum. One branch had an anastomosis to the left branch of portal vein in the liver and the other distributed in the hepatic quadrate lobe. We considered this aberrant left gastric vein to be a congenital residue of the embryological left portal vein. The present case is the third Japanese case to have been described minutely in the literature, following the two cases reported by Miyaki et al. (1987). Persistence of the umbilical vein and the absence of the celiac trunk were also observed.