Our experience with anomalies of the first branchial cleft is reviewed. This includes 38 cases, the largest series collected to date. A new classification is proposed based on the anatomic findings--whether cyst, sinus, or fistula is present. The embryology and pathology of first cleft anomalies are discussed and an approach to the diagnosis and management of these lesions is given. In addition, a patient with a branchial cleft defect involving the middle ear space is reported.