Stridor in a neonate evokes a series of prompt evaluations to determine the etiology. Common etiologies include inherent structural defects (such as laryngomalacia), vocal fold paralysis, acquired infectious etiologies, or extrinsic compressions. We report a neonate with a lymphatic malformation (lymphangioma) initially confined to the larynx that presented with stridor and progressive respiratory failure. Only seven other cases have been reported to present with lymphatic malformation confined solely to the larynx. There are several different modalities available to treat this condition, but recurrences are common. The presentation and management are discussed below. Physicians should be aware that stridor in a neonate can result from this unusual intrinsic obstruction of the larynx.