To explore the etiology, clinical manifestations, diagnosis and treatment of IgG4-related Mikulicz disease and to provide a reference for clinical diagnosis and treatment.

Case data from a patient with IgG4-related Mikulicz disease occurring in the submandibular gland and the literature were retrospectively analyzed.

IgG4-related Mikulicz disease is a rare autoimmune disease. Its typical features include lacrimal gland and salivary gland swelling, a significant increase in the serum IgG4 level (> 1.35 g/L), and IgG4-positive plasma cell infiltration into a large number of pathological tissues. The disease can easily recur. Treatment is mainly symptomatic, including surgical resection and administration of glucocorticoid and other drug treatment. Long-term follow-up is required after the operation.

IgG4-related Mikulicz disease has no specific clinical features. The diagnosis relies mainly on pathological examination and serum IgG4 examination. The main treatment methods are surgery and the use of glucocorticoids, which require long-term follow-up.