Bladder exstrophy is a rare birth defect that typically requires patients to undergo multiple surgical procedures throughout the course of their childhood. Many ultimately undergo operations that use segments of bowel for the reconstruction and/or augmentation of the urinary tract, which imparts an increased risk of malignancy in these patients. We present the case of a 59-year-old man with a history of bladder exstrophy managed with ureterosigmoidostomies revised to an ileal conduit who developed a large adenocarcinoma in the ileal conduit that extended into small bowel, sigmoid colon, and ureter.