Occasionally, a preduodenal portal vein (PDPV) is found in pediatric patients with intestinal obstruction due to duodenal compression. In adults, it is often symptom-less and is an exceptionally rare condition. It is of surgical importance, however, because the vein lies in the most superficial position ventral to the common bile duct and is easily damaged during operations involving the biliary tract and duodenum. We recently treated a patient with PDPV, which stimulated a review of the disease process, including diagnosis, association with other anomalies, and surgical treatment.