Abstract: As a rare type of autosomal recessive inheritanced insulin resistance syndrome，type A insulin resistance syndrome（TAIRS） is caused by mutations in the insulin receptor gene which induces insulin dysfunction and a series of physiological and pathological abnormalities due to defective binding of insulin to its receptors. Rarely diagnosed clinically，TAIRS is mainly characterized by severe insulin resistance，hyperinsulinemia，hyperandrogenism，and acanthosis nigricans. At present there is no explicit TAIRS diagnosis standard，and the diagnosis is mainly based on the clinical symptoms and insulin receptor gene screening. There is no radical treatment means. We just improve insulin resistance and alleviate the symptoms.

Key words: Receptor, insulin, Insulin resistance, Hyperandrogenism, Polycystic ovary syndrome, Type A insulin resistance syndrome