True branchial cleft anomalies are duplications of the membranous part of the external auditory canal, and they manifest clinically as cysts, sinuses, or fistulas. This fault may lead to external auditory canal stenosis and atresia. Because of misdiagnosis, management is often inadequate, recurrence is common and iatrogenic injuries of the facial nerve have been reported. This report features a case of microtia and congenital cholesteatoma with first branchial fistula. external auditory canal stenosis complicated by middle ear and external canal cholesteatoma but branchial fistula, opening in the zygomatic root and a sinus in the helical root may be the cause of this feature. Clinical features are described and the embryological relationship between the first branchial cleft anomaly and external canal atresia is discussed.