A 4-year-old boy was referred to the child psychiatry clinic for management of tantrums with aggression and self-injurious behavior. Tantrums were unpredictable and often resulted in severe head-butting. His parents also reported atypical social and language development, disrupted sleep, and persistent impulsivity with attempts to elope.The patient had a complex medical history that was marked by prematurity at 32 weeks, slow postnatal growth, and severe gastroesophageal reflux. Genetic testing following an episode of hypoglycemia revealed microdeletion of the 16p11.2 region of chromosome 16. His pediatrician documented a normal neurological examination without dysmorphology or congenital anomalies.A developmental evaluation revealed delays in motor function and language development notable by age 2 years. He attended a 0 to 3 program with intensive speech therapy and then moved to a self-contained preschool classroom with a 1:1 staff-to-student ratio. He received school-based speech, occupational, and physical therapies. Testing results revealed clinically significant scores on the Autism Diagnostic Observation Schedule. Cognitive testing results showed that his IQ was below average but was likely an underestimate due to noncompliance. The parents stated that he played repetitively, lining up toy cars and spinning their wheels. He did not seek or give comfort to others when distressed. He had tactile hypersensitivity but pain hyposensitivity, flicked light switches repetitively, and mouthed objects.In the office, the patient showed a minimal response to clinicians' social overtures and poor eye contact. He replayed videos on a smartphone while laughing, spinning around, and flapping his hands. He did not respond to prompts for joint engagement and no imaginative play was observed. When the videos stopped, he repeatedly climbed onto chairs and jumped off. As clinicians attempted to redirect him, he became very agitated and his mother intervened physically to protect him from self-injury. Related article page 514 WHAT WOULD YOU DO NEXT? A. Referral of family members for genetic testing to determine 16p11.2 carrier status B. Pharmacotherapy with R-baclofen based on the mouse model of 16p11.2 deletion C. Pharmacotherapy with citalopram for rigidity and repetitive behavior D. Referral to a behavioral intervention program plus pharmacotherapy with aripiprazole for irritability, agitation, and self-injury Clinical Review & Education