We report a case of a giant bilateral extra-adrenal myelolipoma. To our knowledge this is only the second reported case of either giant or bilateral extra-adrenal myelolipoma and the first in which both features are present. The tumor is also notable for having areas of fibromyxoid degeneration histologically similar to those seen in low-grade fibromyxoid sarcomas. This feature, previously unreported, expands the differential diagnosis of extra-adrenal myelolipomas.