Periauricular cysts, sinuses, and fistulas occur commonly in the pediatric population. They arise from developmental defects of the first branchial cleft and first branchial arch. In most instances the diagnosis and management of these conditions are straightforward, but exceptional presentations sometimes occur. Failure to recognize these unusual cases may result in inadequate treatment and subsequent recurrence, and even if the correct diagnosis is made, surgical management of these lesions may be complicated. A series of 15 cases of periauricular congenital lesions is reviewed, of which three cases illustrating a diagnostic or surgical challenge are presented. The embryology, presentation, and management of these anomalies are discussed. This is one of the largest series of first branchial cleft anomalies reported in the literature, and our paper uniquely discusses first branchial cleft anomalies and preauricular sinuses together, with an emphasis on the surgical management of facial nerve, external ear, and middle ear involvement.