Introduction: Congenital midline cervical clefts (CMCCs) are a rare developmental anomaly of the anterior neck. It is characterized by a midline defect between the mandible and manubrium which typically presents with a superior nipple-like skin tag, an erythematous band of atrophic skin, and a caudal sinus tract. The pathogenesis remains incompletely understood, however, prevailing theories revolve around delays in fusion of the mandibular processes during development of first branchial arch structures. CMCCs remain incredibly rare, showing no predilection for gender or race. The exact incidence is unknown but is thought to be less than 1 in 100,000 live births. Method: We report a case report of a 5 year old boy presenting with a congenital midline cervical cleft that demonstrated all of the typical characteristics described in the existing literature. Surgical excision and repair with multiple small Z-plasties was performed. Histopathological analysis was performed on the excised cervical cleft defect. Conclusion: Congenital midline cervical clefts are rare developmental anomalies of the neck that present a unique diagnostic and treatment challenge. Surgical treatment usually has favourable outcomes, however, must be planned carefully in an area with a high risk of scar contractures and web formation. While there is no treatment consensus given the rarity of the condition, most reports utilize some variation of Z-plasty repair.