Leiomyoma is a benign tumor of smooth muscle origin that is most commonly found in the uterus, gastrointestinal tract, and skin. Intraoral leiomyomas are rare, usually asymptomatic, and, most often present in the fifth decade of life. A review of the literature since 1884 yielded only 125 cases, including 23 of the tongue. We report the first case of a congenital leiomyoma of the tongue presenting with airway obstruction. A 6-day-old female infant, intubated since birth for respiratory distress, was evaluated for an obstructing oropharyngeal mass. The tumor was pedunculated, mucosa-covered, and localized to the posterior tongue. The mass was completely excised using a CO2 laser. There has been no recurrence in 1 year of follow-up.