Insulin autoimmune syndrome (IAS), also known as Hirata disease, was first described by Yukimasa Hirata in Japan in 1970. Disease is a rare endocrine disorder characterized by recurrent attacks of severe hypoglycaemia, characterized by elevated serum insulin, and positive insulin autoantibodies. The diagnosis is very challenging, requiring a careful workup. Early identification and diagnosis of IAS can significantly improve prognosis. Up to now, no standardized guidelines exist for the diagnosis and treatment of IAS.