Inferior vena cava (IVC) occlusion may have widely varying clinical presentations that overlap with congenital IVC anomalies. Nevertheless, appropriate diagnosis, including differentiation from congenital absence, is mandatory. Endovascular therapy of chronic occlusions appears to yield results comparable to those of open repair. We report a case of infrahepatic IVC occlusion misdiagnosed as congenital absence of the IVC. The IVC occlusion was successfully recanalized and treated with angioplasty and stent placement.