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Background  Congenital anomalies of the venous system are known but congenital absence of infrarenal inferior vena cava with absent deep venous system of the lower limbs is extremely rare.    Case presentation  We report the case of an 11-year-old Indian girl who presented with large venous collaterals on her anterior abdominal wall and recurrent non-healing venous ulcers on her left leg with complete absence of infrarenal inferior vena cava and absent deep veins of her lower limbs.    Conclusions  Congenital absence of infrarenal inferior vena cava may occur with absence of the deep venous system of the lower limbs. We have reported this case because of its extreme rarity and to enhance awareness of this entity that has no treatment currently.

Congenital absence of infrarenal inferior vena cava and deep veins of the lower limbs: a case report