The diagnosis of the rare congenital extrahepatic portosystemic shunts is of clinical significance because of the risk of hepatic encephalopathy; liver dysfunction; and associated cardiac, gastrointestinal, vascular, skeletal and genitourinary anomalies. This article describes two varying cases showing the same type of the extrahepatic congenital shunts (Type II). Both the patients were clinically asymptomatic. The first patient initially presented with unprovoked deep venous thrombosis and a staging CT scan was performed to identify any potential underlying malignancy. The second was a polytrauma patient in whom a congenital extrahepatic portosystemic shunt was identified on the CT scan performed to investigate the trauma-related injuries. The first case underwent hepatological investigations, including a fibroscan to rule out liver fibrosis, and was diagnosed as having a Type II congenital malformation, while the second case is under observation post recovery from his traumatic injuries and will be subsequently referred to the hepatology team in the future. Although uncommon, extrahepatic portosystemic shunts can cause significant morbidity and mortality, and all new cases diagnosed radiologically should be further investigated by referring them to a hepatologist.