Immunoglobulin G4-related diseases(IgG4-RD)are chronic, systemic diseases that have received much attention in recent years. IgG4-RD can affect almost all tissues of the body, mainly manifested by swelling and space-occupying changes in the involved sites. It is called IgG4-related ophthalmic disease(IgG4-ROD)when the lesions invade the ocular area. The disease mainly invades the lacrimal glands, orbital fat, infraorbital nerve, extraocular muscles, and eyelids. At present, the main treatment modalities for IgG4-ROD include medication, surgery, and radiation therapy, etc. With the enhanced understanding of the disease and the increasing cure rate in recent years, this article reviews the latest progress in the epidemiological characteristics, clinical manifestations, imaging features, diagnosis and treatment of IgG4-ROD.