Abnormalities of the first branchial cleft are rare. They may present with a cutaneous defect in the neck, parotid region, external auditory meatus or peri-auricular area, or with inflammatory or infective lesions at these sites.

A retrospective case note review of the patients treated by the senior author is presented. This group consisted of 18 patients and represents the largest published UK series to date. Eleven patients (65 per cent) had undergone incomplete surgery prior to referral.

Over half the patients had a clinically apparent lesion in relation to the external auditory meatus. There was a variable relationship between the tract and the facial nerve, which was identified at surgery in 15 cases.

These findings are consistent with those of previously published series. Clinicians should keep this diagnosis in mind when assessing patients with infected lesions in the neck and parotid area. Surgeons should be familiar with parotid surgery, in children where appropriate, and be prepared to expose the facial nerve before embarking on the surgical management of these lesions.

A variant of a type 2 first branchial cleft anomaly, in which accessory ossicles were found, is described. There follows a discussion of the classification of first branchial cleft abnormalities and how this particular case falls outside the standard classification. CT scanning is mentioned as the investigation that is most useful for defining these abnormalities.