Myelolipomas are rare tumors consisting of both adipose and hematopoietic tissue and are typically found within the adrenal gland. Extra-adrenal involvement is rare, especially those tumors involving the perirenal space and collecting system. We report a case of a patient with an incidentally discovered perirenal mass that was initially concerning for a retroperitoneal liposarcoma. Following surgical resection and pathological analysis, the lesion was found to be an extra-adrenal myelolipoma. This case report and review of the literature demonstrates the importance of the proper workup and management of perirenal lipoma variants while addressing the issues of tissue biopsy, surgical intervention, and pre- and post-operative surveillance.