Congenital midline cervical cleft is a rare congenital disease. The disease is often misdiagnosed as a branchial cleft deformity, thyroglossal duct cyst, or other skin diseases. It has the following characteristics: skin defect at the midline of the anterior neck, a skin tag at the upper end of the lesion, and a blind sinus tract at the caudal aspect with or without mucoid discharge. Treatment is usually for aesthetic purposes; therefore, early surgical en bloc resection with Z-plasty or W-plasty is recommended to reduce recurrence and scar formation.