Several of the environmental stimuli suggested to play a role in the pathogenesis of ASD involve altered immune responses during gestation. In this review, we discuss maternal immune activation as a primary risk factor for ASD, with an emphasis on recent findings from animal models of prenatal immune challenges. We further address the presence of autoantibodies as an additional immune-related autism risk factor, drawing upon work done in rodent and monkey models. We then explore the intersection between genetic and environmental susceptibility, with a focus on gene-environment interactions and immune involvement, in genetic risk factors for autism. Finally, we provide emerging evidence for the role of immune dysregulation in the pathogenesis of ASD.