Congenital midline cervical cleft is a rare disorder with a wide range of presentations. To date, there have been fewer than 35 cases reported in the English-language literature. A rarer finding is the association of bronchogenic cysts or respiratory epithelium with the midline cleft. The clinical presentation of congenital midline cervical cleft with a cephalic skin tab, atrophic cleft, and caudal sinus may distinguish it from other conditions of the midline neck. We present an 18-month-old girl with congenital midline cervical cleft to illustrate its clinical presentation and the proper treatment of this condition.
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