Congenital midline cervical cleft is a relatively uncommon malformation of the anterior neck for which most of the current literature comprises case reports. There is a spectrum in clinical features, but the prototypical description is of a midline groove or cleft of atrophic skin with a skin tab at the cephalic end and an inferiorly oriented sinus tract at the caudal end. An underlying dense fibrous cord is usually described. The most widely postulated etiology is impaired midline fusion of the upper branchial arches. Some of the recent case reports advocate for removal of the defect and closure with Z-plasty. Here we report a case of a similar congenital lesion in a male patient. In our case, there was a conspicuous skin tab caudal to which there was a dense fibrous cord running vertically in the midline under the skin documented since the child was first examined at 5 months of age. The skin overlying the fibrous cord was mildly atrophic and only very subtly indented but was not desquamating or edematous. There was no open sinus tract at the caudal end, but within the skin tab, on histology, we did note an inclusion cyst lined by respiratory type mucosa with squamous metaplasia. The cord was composed of fibrous tissue and skeletal muscle, as has been described. In our case, we describe hamartomatous proliferations within the dermis of the skin tab, not previously described, which we feel provides further support to the embryologic closure defect hypothesis. Our case is distinct because the absence of an external cleft allowed for a more conservative surgical approach.