Abstract The fifth reported case of renal myxoma is described. This tumor was removed from a sixty-two-year-old man with a history of hematuria. Diagnostic evaluation had revealed a mass in the lower pole which produced only splaying of the intrarenal vasculature on arteriography. Right nephrectomy was performed. The myxoma was gelatinous in gross appearance and arose from the collecting system. Although these tumors rarely metastasize, a tendency to local recurrence demands their complete removal.