Introduction: Immunoglobulin G4-related disease is a systemic pathology characterized by polyclonal infiltration of IgG4 - bearing plasma cells. Objective: updating the clinical characteristics and complementary tests necessary to make an adequate diagnosis of the Immunoglobulin G4 – related disease, as well as the main lines of currently available treatment. Method: a systematic bibliographic review was carried out in the PubMed, SciElo, Medline, Science Direct, Medigraphic and Scopus databases. The keywords used were: Immunoglobulin G4 -Related Disease; Immunoglobulin G4; Mikulicz Disease and their translations into English. Those with the highest scientific content were selected, for a total of 28 references. Development: few data are available about genetic factors in this disease, which can affect most organs; for example, the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, and lungs. In the oral cavity the associated pathological processes are sialadenitis and Mikulicz Disease. For the diagnosis it is necessary the integration of clinical, serological and imaging data, fundamentally. The most used treatment and with the best response are glucocorticoids. Conclusions: IgG4 -Related Disease is a systemic entity that causes autoimmune pancreatitis, retroperitoneal fibrosis, and lymphadenopathy. Sialadenitis and Mikulicz disease occur in the mouth. It is necessary to make a correct diagnosis in order to apply an adequate treatment, according to the lines currently available
