Reports of primary small intestine malignancies are rare. Even more uncommon is primary carcinoma in an ileal conduit. Here, we report a case of primary adenocarcinoma in an ileal conduit that developed 14 years after radical cystectomy and diversion to an ileal conduit for transitional cell carcinoma of the bladder. To our knowledge, only one case of primary adenocarcinoma developing in an ileal conduit after a radical surgery for bladder cancer has been reported previously.