Congenital atresia of external auditory canal is a rare congenital deformities arising from the ectodermal origin, embriologically. To author's knowledge, this is the first presented case in our country about congenital atresia of external auditory canal with chronic mastoiditis. A 26-year-old housewife had been sufferring from atresia of external auditory canal and hearing impairment on left ear since birth according to the parents and pus discharge from left post auricular region with pain for the 5 years. Radiological examination suggested chronic mostoiditis. Modified radical mastoidectomy and meatoplasty was performed under general anesthesia.