Extra-adrenal myelolipomas are exceedingly rare benign tumors composed of adipose and myeloid tissues, which have been reported to occur in various sites including the retroperitoneum, pelvis, and thorax. Myelolipomas are more commonly encountered in the adrenal glands. We illustrate a case of a 72-year-old woman with surgically proven bilateral perirenal and para-aortic lymph nodal myelolipomas detected incidentally by computed tomography. Extra-adrenal myelolipomas can be difficult to distinguish from other fat-containing lesions particularly liposarcomas, which are more commonly encountered in the retroperitoneum. This case highlights the unusual multifocal involvement of extra-adrenal myelolipomas and despite its rare occurrence, should be included in the differential diagnosis of retroperitoneal lipomatous lesions.