Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 3 4 2020 12 01 236 240 Nasrin Moazzen Clinical Research Development Unit of Akbar hospital, Mashhad University of Medical Sciences, Mashhad, Iran Hamid Ahanchian Clinical Research Development Unit of Akbar hospital, Mashhad University of Medical Sciences, Mashhad, Iran Samaneh Norooziasl Department of Pediatric Endocrinology, Mashhad University of medical sciences, Mashhad, Iran Hamid Reza Kianifar Clinical Research Development Unit of Akbar hospital, Mashhad University of Medical Sciences, Mashhad, Iran 2021 07 17 There are more than 400 different primary immune deficiencies worldwide. Amongst them, patients with humoral immunodeficiency are more common. Most of the innate immune defects, affect the phagocytic system. There are a few cases of toll-like receptor deficiency with innate immune defects, like TLR3 mutations, which usually present with Herpes simplex encephalitis. Herein, we report a two-year old boy with TLR3 deficiency, who was presented with recurrent infections and type one diabetes mellitus. https://igj.tums.ac.ir/index.php/igj/article/view/82 https://igj.tums.ac.ir/index.php/igj/article/download/82/59