Congenital aural atresia is a relatively common congenital deformities arising from the ectodermal origin. It is observed much more often unilaterally and on the right side. Reconstruction surgery for aural atresia should be performed when the child is about 17 or 18 years in unilateral aural atresia, and when about 6 or 7 years old in bilateral atresia. Even if unilateral aural atresia, congenital aural atresia combined cholesteatoma can develop a infected auricular fistula, Bezold's abscess, facial paralysis, perilymph or lateral semicircular canal fistula, cerebrospinal fluid and meningitis, corrective surgery is performed regardless of the patient's age for prevention of other complications. The authors have recently experienced two cases of congenital aural atresia associated cholesteatoma and report with reviews of literatures. We experienced two cases of unilateral congenital aural atresia, which were combined with facial nerve palsy and postauricular fistula due to cholesteatoma. In these cases, we got a hearing improvement and normalized facial nerve function through the external canaloplasty and tympanoplasty.