A case of infrahepatic interruption of inferior vena cava with hemiazygos continuation into a persistent left superior vena cava and partial visceral heterotaxia in association with an otherwise normal heart is presented. The peculiarity of this rare lesion without cardiac malformation and the possible explanation for it are discussed. The clinical significance of systemic venous anomalies is discussed.The association of complex cardio¬ vascular anomalies with partial heterotaxia,13 and with anomalies of the systemic venous connection,46 has been well documented. We found a case with both incomplete visceral in¬ version (partial heterotaxia) and sys¬ temic venous anomalies without any associated cardiac malformation. The purposes of this paper are to present the details of this case, to outline the possible explanation for this associa¬ tion, and to discuss briefly the clinical applications of knowledge of anom¬ alies of the systemic venous connec¬ tions.A 5-year-old white girl was admitted to Good Samaritan Hospital in April 1969 for cardiac catheterization. A heart murmur had been noticed in her infancy. No history of cyanosis or dyspnea was present. She had been a healthy child with normal growth and development. Because of a uri¬ nary tract infection at the age of 4 years, an intravenous pyelogram was obtained, at which time residual barium in the appen¬ dix was noticed (from a previous cardiac series with barium swallow) on the left side of the abdomen (Fig 1, top).