Congenital macroglossia is associated with a variety of syndromes, most commonly Down syndrome and Beckwith-Widemann syndrome. Clinically, macroglossia may result in respiratory compromise, dysphagia, and poor cosmesis. A variety of treatments have been proposed, with surgical resection being the most common. We review management and describe a new surgical technique, consisting of a keyhole resection to decrease the width and length of the tongue. Between 1990 and 1992, five keyhole resections for congenital macroglossia were performed on four consecutive children at Texas Children's Hospital, Baylor College of Medicine, Houston. Patients had improved cosmesis and improved function of the oropharyngeal airway, with no change in speech and feeding. Keyhole resection is an effective treatment for macroglossia.