Autism Spectrum Disorders (ASDs) are neurodevelopmental conditions characterized by impairments in social interaction and repetitive behavior. Recent studies suggest that de novo mutations play a pathological role in ASDs, highlighting the importance of analyzing the functional consequences and their impact on individual phenotypes of these mutations. In this study, we focused on POGZ (Pogo transposable element with ZNF domain), a gene product, on which many ASDs-associated mutations have been identified. We generated a mouse model heterozygous for a Y594C mutation in POGZ (Y594C mice), which identified in patients with ASDs and found that Y594C mice showed ASDs-related behavioral abnormalities, including impaired social behavior. We previously generated a mouse model heterozygous for a Q1038R mutation in POGZ (Q1038R mice), which identified in patients with ASDs and found that Q1038R mice also showed ASDs-related behavioral abnormalities as seen in Y594C mutant mice. Interestingly, while Q1038R mice exhibited developmental abnormalities, such as smaller brain, Y594C mice had a generally normal brain size and structure. Comprehensive research using these model mice will help to unravel the molecular and cellular pathogenesis of de novo POGZ mutations in ASD.