Renal medullary carcinoma is rare and extremely aggressive neoplasm that typically affects young patients of African decent who demonstrate sickle cell trait or disease. Since the original description in 1995, only few cases have been reported outside the United States. A 29 year-old Canadian male of Afro-Caribbean decent with sickle cell trait developed right-sided hemiparesis due to brain infarct. During the clinical work-up, a 3 cm renal tumor was detected in a horseshoe kidney. The patient died suddenly 2 weeks after the presentation of massive non-neoplastic pulmonary thromboembolism, confirmed at autopsy. The final diagnosis of renal medullary carcinoma was established after the autopsy. Due to the small size of the tumor and the limited metastatic spread only to the regional lymph nodes, the tumor was considered an incidental finding, and not the primary cause of patient?s death.