A 6-year-old girl was referred to our department due to pyelonephritis. Voiding cystourethrogram (VCUG) revealed grade 4 vesicoureteral reflux (VUR) and urethral deformity (stenosis and lateral deviation). She had a history of sacrococcygeal teratoma resection in the newborn. Urodynamic study revealed a large-capacity hypotonic bladder and poor bladder emptying. Magnetic Resonance Imaging of the spine demonstrated no abnormal findings. Despite conservative therapy, there were no improvement of VUR. Then, urethral dilation and anti-reflux-surgery were performed. Six months after the operation, VCUG showed no VUR. However, she has persistent residual urine due to neurogenic voiding dysfunction, and is being treated with a regimen of frequent timed voiding to reduce urinary residual and urinary tract infection.