We describe the complex presentation of a patient with renal medullary carc
inoma, a newly described entity primarily affecting young patients with sic
kle cell trait. Renal medullary carcinoma is an aggressive, rapidly destruc
tive tumor associated with a delayed diagnosis and a poor outcome. The most
common presenting signs and symptoms include hematuria, abdominal or flank
pain, and weight loss. Sickle cell trait as the sole cause of hematuria in
young black patients is a diagnosis of exclusion. Hemoglobin electrophores
is, intravenous pyelography, and computed tomography scans should be the mi
nimal studies performed in young black patients with hematuria.