Megalourethra is a very rare congenital anomaly of penile urethra. Herein, we report a case of scaphoid megalourethra and reviewed the literature. Antenatal ultrasonographic evaluation revealed bilateral hydroureteronephrosis. On postnatal physical examination, penile shaft was found to be dilated ventrally which became more evident during micturition. Bilateral hydroureteronephrosis persisted on postnatal ultrasonograhic examinations. Voiding cystourethrography did not reveal out vesicoureteral reflux. On cystourethroscopy, anterior urethra was found to be extremely dilated. Due to recurrent breakthrough infections, vesicostomy was performed after 2.5 months. After vesicostomy, upper urinary dilation progressively regressed; and reduction urethroplasty, urinary undiversion, and circumcission were performed at postnatal 8 months. Bladder capacity was found to be normal without post- void residual on voiding cystourethrography. As a conclusion, megalourethra should be considered in the differential diagnosis of penile deformity.