An 8-year-old girl was born with crossed fused renal ectopia and neurogenic bladder due to sacral agenesis. Due to progressive upper tract deterioration and incontinence despite clean intermittent catheterization and pharmacotherapy with anticholinergic agents, the patient underwent augmentation colocystoplasty at the age of 4 years. Four years after surgery the girl was readmitted because of persistent febrile urinary tract infection, persistent metabolic acidosis, and intermittent watery diarrhea. A cystogram revealed a fistula between the dome of the augmented bladder and the transverse colon. The fistula was successfully resected. The presence of enterovesical fistula should always be suspected in a patient with augmented bladder who have late onset of urinary tract infection, metabolic acidosis, and diarrhea.