The inferior mesenteric vein is one of the tributaries of the portal vein which receives blood from the large intestine, sigmoid colon and the rectum. It usually connects with the splenic vein before draining into the portal vein. It may however show variations such as joining the superior mesenteric vein directly or to the confluence of the superior mesenteric vein and splenic vein. Varices of the inferior mesenteric vein can be seen in the presence of portal hypertension due to various reasons and this can be isolated or in association with other portosystemic collaterals.We report a case of a giant isolated inferior mesenteric vein with an anomalous insertion. The inferior mesenteric vein was seen to be connected to the superior mesenteric vein before draining into the portal vein. Further, on the distal end it was seen to be connected to the left renal vein, which in turn had dilated and enlarged. The left testicular vein was observed draining in to the inferior mesenteric vein but was not enlarged. The superior mesenteric vein, splenic vein and portal vein were not enlarged or varicosed even though the liver showed features of cirrhosis with no obvious signs of splenic enlargement. This report contributes to enhance the knowledge on a rare anatomical connection between portal system and the systemic circulation and will be important to surgeons operating in this area.