A 30-month-old girl presented with gastrointestinal symptoms and a febrile urinary tract infection. Sonographic and radiographic imaging demonstrated left hydronephrosis due to a radiolucent ureteral stone. Surgical exploration identified an aberrant, patent left umbilical artery causing ureteral obstruction. Stone and urine analyses revealed hereditary xanthinuria that had not previously been recognized in this child and her family.
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