Abstract: Three cases of IgG4-related skin disease were reported and he related literatures were reviewed. All the three cases in our study had uniform clinicopathological manifestation and high level serum IgG4. The clinical presentations were pruritic infiltrated nodules or plaques on the cheek, eyelid, periauricular region, neck and chest, together with related symptoms of lacrimal glands and parotid glands. Histopathology of the lesions revealed infiltration of lymphocytes and plasma cells in the dermis and subcutaneous tissue, including abundant IgG4-positive plasma cells. Glucocorticoid remain the most effective management for IgG4-related skin disease.