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Absence of inferior vena cava (IVC) is an uncommon congenital abnormality with few clinical repercussions. We report the case of a 39 year old man with chronic pelvic pain, in whom a macroscopic hematuria episode occurring during exercise led to the discovery of an echographic pelvic venous stasis syndrome. Abdominal and pelvic computed tomography scanning then magnetic resonance imaging of inferior vena cava revealed absence of the postrenal segment of IVC with azygos continuation and considerable collateral venous derivations, leading to pelvic cavernoma.