“…1 It has an incidence of 1 per 1000-1500 live newborns, it occurs more frequently in males with a 3:1 ratio, the age at the time of diagnosis is between 20 to 40 years and it is considered an exclusive right-sided pathology, except in exceptional cases, caused by abnormal embryological development of the renal vasculature. [2][3][4] It is associated with other congenital anomalies such as horseshoe kidney, renal agenesis, renal hypoplasia, Goldenhar syndrome (first and second branchial arch syndrome), hypospadias, intestinal malrotation, syndactyly, turner syndrome, myelomeningocele, among others. 2 The inferior vena cava comes from three venous systems: posterior cardinal, supracardinal and subcardinal 5 .…”