Rett syndrome is a severe neurodevelopmental disease with a prevalence of 0.88 per 10,000 girls aged 5-18 years, and is often caused by mutations in methyl-cytosine-phosphate-guanine (CpG)-binding protein 2. Disorder of respiratory control is a prominent feature of Rett syndrome. Brainstem serotoninergic neurons are known to play an important role in the arrangement of breathing rhythm and pattern. We present a patient whose severe hyperventilation and apneic attacks resolved with the concomitant treatment of fluoxetine and buspirone. To our knowledge, we describe the first patient with Rett syndrome to receive fluoxetine for respiratory problems.