Uretero-inguinal hernia (UIH) is a rare condition in children. We present our experience with an unexpected UIH in a male infant with associated anomalies and a literature review of pediatric cases. A full-term male newborn was referred to our hospital for microcolon and right hydroureteronephrosis. Voiding cystourethrography showed grade-IV right vesicoureteral reflux and a dilated, convoluted, ureter protruding in the right inguinal canal, suggesting a right UIH, which was uneventfully repaired at 2 months of life. UIH is a rare type of inguinal hernia in children that pediatric urologists and surgeons should be aware of. In experienced hands, and when preoperatively suspected, surgical management is safe and effective.