Young patients with sickle cell trait or disease present a higher risk for renal medullary carcinoma, an aggressive renal tumor, with dismal prognosis, with a median survival of 4 months from the time of diagnosis. We report a 26-year-old patient with a 2-month history of bone pain at the right iliac crest, loss of weight, recurrent macroscopic hematuria and abdominal mass. Imaging studies demonstrated a 10-cm mass in the right kidney. Pathological evaluation revealed a medullary carcinoma and the patient died after 4 months, despite adjuvant treatment. Whenever a patient with sickle cell trait or disease presents with hematuria and/or flank pain, the possibility of a renal medullary carcinoma should always be kept in mind, with thorough investigation and expeditious treatment.