A first branchial cleft anomaly is an unusual clinical condition and t
he clinical picture is similar to that seen in other much more common
diseases. This combination may result in a diagnostic delay and insuff
icient and dangerous primary surgery, resulting in facial nerve damage
. This paper reviews the embryological background of first branchial c
left anomalies, the varying clinical presentation and the surgical tre
atment, illustrated by three case histories. A revision of the most co
mmon classification system is proposed.