First branchial cleft sinus presenting with cholesteatoma

Disorders of the first branchial cleft are rare and often present difficulties in diagnosis. A case is reported that presented with the clinical and histological features of cholesteatoma. The embryology, diagnosis and treatment of branchial arch defects are also reviewed. The case highlights the fact that a high index of suspicion should be aroused for patients with a discharging ear in the presence of an intact tympanic membrane.