Recurring hematuria revealing absence of infrarenal segment of the inferior vena cava

Abnormal development of the inferior vena cava is a relatively rare condition. We report the case of a 47-year-old woman presenting with an absent infrarenal segment of the inferior vena cava revealed by recurring episodes of gross hematuria. This entity probably resulted from perinatal acquired thrombosis rather than from a congenital anomaly and is associated with compensatory dilation of collateral venous pathways. This extremely rare hemorrhagic presentation is presumably caused by rupture of small dilated renal or vesical veins. Ignorance of this atypical presentation can lead to erroneous or delayed diagnoses.