Congenital midline cervical cleft (CMCC) is a rare developmental defect of the anterior neck normally characterized by an atrophic mucosal plaque with a cranial nipple-like skin tag, a short caudal sinus, and may be attached to a subcutaneous fibrous cord of variable length. Clinically, patients present at an early age with, white females being the most commonly affected population. In addition to aesthetic concerns, CMCC can prevent full extension of the neck, result in micrognathia and torticollis, predispose patients to infection, and can coexist with other clefting defects or cysts. Fewer than 50 cases have been published in the English-language literature. Herein, we report a case of CMCC that also presented with a mild contracture of the right sternohyoid muscle. The embryopathogenesis, histopathology, diagnosis, and treatment of this rare condition are also discussed.