Congenital midline cervical cleft (CMCC) is an extraordinarily rare midline lesion that is present at birth. The lesion can cause micrognathia and cervical contracture via underlying traction on the mandible. It is essential to recognize the condition and excise the lesion within 1 year of diagnosis to mitigate long term cosmetic and functional problems. We report a 10-month-old male infant with CMCC diagnosed immediately after birth. Ultrasound with color doppler imaging confirmed the anatomic characteristics of the lesion. The lesion was surgically excised and closed in layers, with no postoperative complications and good functional and cosmetic results. The pathognomonic presentation, etiology, differential diagnosis, and optimal surgical approach are also discussed.