Tonsil is a part of the immune system and its abnormal immune function is closely related to autoimmune diseases. Tonsillectomy has been proven to have good clinical effects in the treatment of some autoimmune diseases, and it is expected to be a supplementary treatment in the comprehensive diagnosis and treatment of some autoimmune diseases. This article reviews the therapeutic effect and related mechanism of tonsillectomy in several autoimmune diseases such as IgA nephropathy, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection and palmoplantar pustulosis.