We report the death of a 17-year-old male exstrophy patient from adenocarcinoma arising at the ureterosigmoidostomy. This was detected at reconstructive surgery at the age of 16 years, 13 years after ureterosigmoidostomy. This case highlights the importance of including children in endoscopic surveillance. We recommend annual sigmoid–colonoscopy commencing 10 years after ureterosigmoidostomy irrespective of age.