This is a report of a case of a rare congenital midline cervical cleft and a description of the surgical approach.

Congenital midline cervical cleft is a very rare developmental anomaly. It represents a failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Associated clinical features could include mandibular spurs, cleft mandible, microgenia, thyroglossal cyst, cleft lip or bronchogenic cysts.

The authors present a case of a midline cervical cleft that was diagnosed and managed at an early age. They discuss the clinical presentation and embryological development of this rare condition. The operative findings, surgical excision and repair of the long vertical defect by Z-plasty are discussed in detail.

The authors report a very satisfactory result following excision and Z-plasty closure of this rare congenital anomaly.