Introduction:Anterior urethral valve (AUV) is a rare congenital anomaly that can cause obstructive uropathy. AUV can lead to variable urinary tract symptoms, and if left untreated, it can result in end-stage renal disease. AUV should be immediately evaluated and managed. Herein, we present a neonate with AUV and diverticulum, who was treated by cystoscopy. Case report:A 11-day-old boy was referred to the nephrology department for evaluation of urinary tract infection, which was developed 2 days prior to admission. Pregnancy and routine ultrasound (US) were normal, and urinalysis showed pyuria. Routine investigations included blood urea nitrogen, serum creatinine, electrolyte panel, urine analysis (UA), and urine culture (UC). Abdominal US examination displayed a normal bladder with normal kidneys. A voiding cystourethrogram (VCUG) showed an anterior urethra diverticulum with dilation of the proximal urethra and trabeculated bladder; however, no vesicoureteral reflux was observed. Finally, the patient underwent endoscopy. Discussion: AUV is a congenital mucosal fold, located distally to the membranous urethra. AUV can be located anywhere distal to the membranous urethra and is found in bulbar urethra (40%), penoscrotal junction (30%), pendulous urethra (30%), and occasionally in the fossa navicularis, respectively. The exact etiology of this disease is unclear. AUV with associated diverticulum is a rare congenital urethral anomaly that can lead to penile swelling, urethral obstruction, urinary retention, incontinence, nocturnal enuresis, bladder rupture, and end-stage renal disease. Depending on the severity of anatomical obstruction, this condition may present soon after birth or later during childhood. VCUG is the most important imaging technique for the evaluation of urethral abnormalities. Congenital AUV in children has a generally good prognosis, but may occasionally result in poor renal outcomes such as renal insufficiency, renal failure, and patient‘s death.