A 1-year-6-month-old Japanese girl with Hinman syndrome manifested urosepsis and severe obstructive nephropathy. Her voiding cystourethrogram (VCUG) revealed high-grade vesicoureteral reflux with hydronephrosis; urodynamic study was compatible with detrusor-sphincter dyssynergia. She was treated conservatively, including clean intermittent catheterization. At 3 years old, bladder function had not improved, and estimated creatinine clearance was in the subnormal range. Hinman syndrome is a potential cause of acute and chronic renal failure in infancy. Taking account of the possibility of this condition in any neonates or infants who present urinary tract infection (UTI) appears to be necessary, since early recognition and proper management of this syndrome may prevent serious complications.