Periauricular cysts, sinuses, and fistulas occur commonly in the pedia
tric population. They arise from developmental defects of the first br
anchial cleft and first branchial arch. In most instances the diagnosi
s and management of these conditions are straightforward, but exceptio
nal presentations sometimes occur. Failure to recognize these unusual
cases may result in inadequate treatment and subsequent recurrence, an
d even if the correct diagnosis is made, surgical management of these
lesions may be complicated. A series of 15 cases of periauricular cong
enital lesions is reviewed, of which three cases illustrating a diagno
stic 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 lite
rature, and our paper uniquely discusses first branchial cleft anomali
es and preauricular sinuses together, with an emphasis on the surgical
management of facial nerve, external ear, and middle ear involvement.