Congenital malformations of the branchial arches are not infrequent. Among them first branchial cleft anomalies are common and they are to remain asymptomatic. The case reported is one of symptomatic fistula auris, which at the beginning led us to the initial diagnosis of an orbital cellulitis, secondary to ethmoidal sinusitis. We have made a review of the literature dealing with these anomalies and we expose in the paper an update perusal of the clinical features and the management of this rare complication.

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