Bladder malignancy in patients with congenital bladder anomalies who have undergone bladder augmentation is a rare but well-recognized condition. These patients present with locally advanced or metastatic disease and have poor survival. We report a case of a patient with myelomeningocele who was incidentally found to have a high-grade intestinal type adenocarcinoma of her bladder augment at the time of cystolithotomy. This case highlights the need to continue to follow patients with congenital bladder anomalies and highlights the lack of adequate screening methods available.
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