A review of the embryology, pathophysiology semiotics and surgical treatment of malformations of the inferior vena cava is given based on 3 clinical histories. The cases consisted of a typical membrane occlusion, an atresia of the infrarenal segment and of a hitherto not described combination of several malformations of the inferior vena cava: membrane occlusion, aplasia of the infrarenal segment of the vena cava inferior and multiple aneurysms of the iliac vein. The symptomatology of malformations of the vena cava inferior depends on the compensation by the collateral or the persisting embryonic veins, respectively, and on the localization and the degree of the obliteration. The varying hemodynamic reactions are described. The following surgical methods for the treatment of inferior vena cava occlusions in the hepatic segment are available: The so called conservative interventions for the creation of collaterals, the direct or indirect recanalization and the bypass operation. Congenital infrarenal atresias of the vena cava are corrected by homologous or prosthetic interposition. The venous replacement with a bovine heterograft presented here, has not yet been described in the literature.