ACTA1 and pulmonary arterial hypertension: Care should be taken to prevent respiratory failure after surgery.<h4>Patient concerns</h4>A 35-year-old man with NM, who had difficult airway, restrictive ventilatory pattern, and pulmonary hypertension, required general anesthesia for surgery because of limited mouth opening.<h4>Diagnoses</h4>The patient was diagnosed with NM (ACTA1 mutation) and coronoid hyperplasia.<h4>Interventions</h4>Awake fiberoptic nasal intubation was performed following preparations for analgesia.