First branchial cleft anomalies are extremly rare and usually associated with the pediatric population, thus constitue a mojor diagnostic problem among adults. First branchial cleft anomaly type I is limited to the external auditory canal and typically occures in adults. Such a dignosis should always raise a suspition of associated cholesteatoma of the external auditory canal (EAC) due to the presence of mechanical barrier in self-cleaning of EAC, which leads to entrapment of epidermal cells and promotes the formation of cholesteatoma.