Inferior vena cava agenesis (IVCA) is an uncommon congenital anomaly which was first described in the seventeenth century. The detection of such an anomaly is often incidental and patients are asymptomatic until the third to fourth decade of life. Due to the scarcity of these cases, there is no consensus on the subsequent treatment and surveillance of incidental asymptomatic IVC anomalies. Herein, we report on a young male with incidental findings of suprarenal IVC agenesis identified on contrast enhanced computed tomography (CT) scan of the abdomen in the course of treatment for acute appendicitis.