Congenital midline cervical cleft (CMCC) is a rare congenital anomaly of the ventral of the neck. The CMCC consists of a skin tag, mucosal surface, and a caudal sinus in the ventral midline of the neck. Embryologically, the
development of CMCC is thought to be mainly due to failure of the branchial arches to fuse in the midline, resulting in a ventral midline defect of the skin of the neck. We report a rare case of congenital midline cervical
cleft in a 6-year old girl, plus a review of the related literature.