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Duplication of inferior vena cava is an uncommon abnormality and is important in daily today practice for vascular surgeons, radiologist and urologist especially during retroperitoneal surgeries and treatment of thromboembolic disease. Radiologically, Duplicated IVC can be mistaken for lymphadenopathy or left pyeloureteric dilatation. Crossed fused kidney with a single ureter defy the embryological theory of ureteric bud crossing the opposite side and induce nephron formation associated anomaly of Duplication of inferior vena cava and malrotation of gut are not reported in a same patient. On meticulous search of literature no such combination of abnormalities has been reported. In this case report we bring forward this rare type of combination of three congenital malformations that is Duplication of IVC, crossed fused kidney and malrotation of gut.