Autism is a neurodevelopmental disorder, characterized by poor social interaction and communication impairment and repetitive behavior. Autism is considered as a genetic and multifactorial disorder, with diverse risk factors involved.Herein, we report a 13-year-old male with common variable immunodeficiency (CVID), who was diagnosed with autism at the age of 3 years old. As there are some evidences about the role of the immune system defects in the pathogenesis of autism, specific primary antibody deficiency diseases such as CVID might predispose some affected cases to such neurodevelopmental disorders.