Chronic granulomatous disease is a rare disorder of phagocytic cell oxidative metabolism. Patients have recurrent infections with catalase positive organisms and granulomatous lesions throughout the body. The genitourinary tract can be an occult site of involvement. We reviewed the prevalence of urological abnormalities in 60 patients with chronic granulomatous disease. Chart review revealed that 23 of the 60 patients (38%) had urological manifestations. Seven patients had 10 ureteral strictures. Treatment of the granulomatous strictures with steroids caused at least a temporary decrease in size or a resolution of the granulomas and strictures with steroids caused at least a temporary decrease in size or a resolution of the granulomas and relief of the obstruction. Three patients had bladder granulomas and 1 had a urethral stricture. A total of 12 patients had urinary tract infections and 7 had altered renal function. Ten patients had other manifestations of genitourinary disease. Abnormalities in the urinary tract are relatively frequent. Risk factors include X-linked disease and low percentile weight. Genitourinary manifestations can cause significant morbidity in chronic granulomatous disease patients. A variety of therapies, including judicious use of steroids and gamma-interferon, are proving to be useful in the treatment of this disorder and in some cases they may offer a nonoperative alternative for relief of chronic granulomatous obstructive disease in these patients.