Introduction. Congenital interruption of the inferior vena cava is a rare entity. It can be associated with other organ and system malformations, especially the cardiovascular system. Usually, patients are asymptomatic and the anomaly is therefore diagnosed incidentally. In some cases it can be of clinical importance. Case Outline. A 22-year-old female was diagnosed during evaluation of cardiac symptoms. Clinical examination showed normal findings. CT angiography and cavography were used to establish the diagnosis of inferior vena cava interruption. Since there was no need for surgical intervention the patient was discharged in good condition. During a five year follow-up no significant health problems were noted with patient leading normal life. Conclusion. Diagnostic tools used to establish the diagnosis of inferior vena cava interruption are ultrasonography, as well as CT and MR angiography and phlebography. Mediastinal masses found on chest roentgenogram must raise suspicion of inferior vena cava interruption. Although the anomaly is rare, physicians should be aware of the infrahepatic interruption of the IVC, especially because it can present at any age.