A broad spectrum of congenital anomalies and pathologic conditions can affect the inferior vena cava (IVC). Most congenital anomalies are asymptomatic; consequently, an awareness of their existence and imaging appearances is necessary to avoid misinterpretation. Imaging also plays a central role in the diagnosis of Budd-Chiari syndrome secondary to membranous obstruction of the intrahepatic IVC. Primary malignancy of the IVC is far less common than intracaval extension of malignant tumors arising in adjacent organs, and imaging can accurately help determine the presence and extent of tumor thrombus, information that is crucial for surgical planning. However, the radiologist should be aware that artifactual filling defects at computed tomography and magnetic resonance imaging can mimic true thrombus in the IVC and must be able to differentiate true from pseudo filling defects. Other imaging findings such as flat IVC and early enhancement of the IVC are useful in limiting the differential diagnosis. Familiarity with the imaging features of the various congenital and pathologic entities that can affect the IVC is paramount for early diagnosis and management.