Myelolipoma of the kidney is a very rare benign disease which is composed of mature adipose tissue admixed with mature hematopoietic elements in varying proportions. The most common location of myelolipoma is in the adrenal glands, but extra-adrenal myelolipomas in other locations such as the retroperitoneum, presacral region, pelvis, and mediastinum has also been reported. In this case report, we present a case of huge extra-adrenal myelolipoma in renal parenchyma which is rare site for its origin. We report a case of extra-adrenal myelolipoma occurring in the left kidney of a 46-year-old Indian man. We describe the radiological and clinicopathologic features of this unusual tumor with a review of the literature. This case is noteworthy because the tumor was very enlarged and its site was unusual. It is generally impossible to distinguish extra-adrenal myelolipoma from other retroperitoneal tumors by radiological imaging given the rarity of tumor more cases need to be reported to elucidate its biological behavior.