We report a case of interrupted inferior vena cava (IVC) as a rare developmental defect. Inferior vena cava interruption is usually accompanied with azygos and hemiazygos continuation, and is asymptomatic. Consequently, venous blood from the caudal part of the body reaches the heart via the azygous vein and superior vena cava. A 50 year old female who came for routine health check-up was found to have pulmonary hypertension on two dimensional echocardiography. On further investigations she also had restriction on pulmonary function test. When computed tomography pulmonary angiography was done, showed dilated azygous vein without pulmonary embolism. Computed tomography of the abdomen demonstrated interrupted inferior vena cava. Such patients are at increased risk of deep vein thrombosis and pulmonary embolism.