OBJECTIVE To identify the clinical and anatomical presentations and to discuss the guidelines for surgical management of anomalies of the first branchial cleft. METHODS Retrospective study. METHODS Three tertiary care centers. METHODS Thirty-nine patients with first branchial cleft anomalies operated on between 1980 and 1996. METHODS All patients were treated surgically. Complete removal of the lesion required superficial parotidectomy with facial nerve dissection in 36 cases. The relationship of the facial nerve and anomalies is discussed. RESULTS Anatomically, 3 types of first branchial cleft anomalies are identified: fistulas (n=11), sinuses (n=20), and cysts (n=8). Clinically, 3 types of presentation are noted: chronic purulent drainage from the ear (n=12), periauricular swelling in the parotid area (n=18), and abscess or persistent fistula in the neck located above a horizontal plane passing through the hyoid bone (n=21). A membranous attachment between the floor of the external auditory canal and the tympanic membrane was observed in 10% of cases. The facial nerve was located lateral to the anomaly in 39% of cases. CONCLUSIONS Before definitive surgery, many patients (n=17) underwent incision and drainage for infection owing to the difficulties in diagnosing this anomaly. Wide exposure is necessary in most cases, and a standard parotidectomy incision allows adequate exposure of the anomaly and preservation of the facial nerve. Complete removal without complications depends on a good understanding of regional embryogenesis, a knowledge of the circumstances surrounding discovery, an awareness of the different anatomical presentations, and a readiness to identify and protect the facial nerve during resection.