First branchial cleft anomalies are congenital rare lesions that can sometimes be difficult to diagnose. During the normal embryonic development the outer ear canal derives from the first branchial cleft. Abnormal development can result in production of a cyst, sinus or fistula with recurring infections. Early and correct diagnosis is necessary for the correct choice of surgical set-up in which identification and preservation of the facial nerve is an important step. A case of first branchial cleft sinus is presented with further discussion of classification, diagnostics and treatment.
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