Three children (2 boys and 1 girl) with exotropia were diagnosed with AS based on their clinical futures: poor ability of verbal expression, a wide-based gait, spontaneous laughter, overactive behavior, excitability, and short attention span. The age of these patients ranged from 31 months old to 9 years old. All clinical information of AS children with strabismus was retrospectively reviewed, and parental consent for the publication of this case report was obtained. The deletion testing on the chromosome at 15q11AS-related regions was performed by multiplex ligation-dependent probe amplification (MLPA) with the genetic DNA extracted from the blood of the patients and their parents. Focus was given on the clinical features of the ocular findings and ocular motility. All AS patients underwent lateral rectus recession surgery with the assistance of intravenous combined inhalation anesthesia.