To determine the risk of bladder cancer following enteric bladder augmentation.Patients followed for care after an enteric bladder augmentation have been entered into a registry; individuals followed for a minimum of 10years were evaluated.The study criteria were met by 153 patients. Indications for bladder augmentation were neurogenic bladder in 97, exstrophy in 38 and posterior urethral valves in 18. There was a median follow-up interval of 27years (range 10–53). A total of seven cases of malignancy developed. Median time to tumor development following augmentation was 32years (range 22–52). Two patients with neurogenic bladder developed transitional cell carcinoma; both were heavy smokers (>50 pack per year history). Two patients with a history of posterior urethral valves and renal transplantation developed adenocarcinoma of the enteric augment. Three patients with bladder exstrophy developed multifocal adenocarcinoma of the augmented bladder. Two patients remain alive, 5 and 6years following radical cystoprostatectomy; five died of cancer-specific causes.Malignancy following enteric bladder augmentation arose in 4.5% (7/153) of our patients and was associated with coexisting carcinogenic stimuli (prolonged tobacco/chronic immunosuppressive exposure), or alternatively with the inherent risk of malignancy existing with bladder exstrophy.