Mucoepidermoid carcinoma (MEC) of the larynx is one of the rarest tumors in the pediatric age group. Our review of the English-language literature found only 1 previously reported case of a laryngeal MEC in a child. We present what to the best of our knowledge is the first case of a pediatric MEC of the larynx with transglottic involvement. Our patient was a 12-year-old girl who presented with long-standing hoarseness and recent dysphagia, breathing difficulty, and pain radiating to the left ear. Investigation revealed the presence of an endophytic mass in the larynx. Histopathologic examination of biopsy tissue identified the mass as a low-grade MEC. The patient was scheduled to undergo a total laryngectomy 1 week later, but she did not report for surgery. Three weeks later, she presented to the emergency department in a semiconscious state and in respiratory distress. Despite all resuscitative measures, she died.