IgG4-related ocular disease, a chronic systemic disease with lgG4, is characterized by elevated serum levels of lgG4 and a dense lymphoplasmacytic infiltrate rich in lgG4-positive plasma cells in involved ocular adnexa such as lacrimal glands, extraocular muscles, infraorbital nerve and so on. It is often the first symptom of systematic disease. IgG4-related ocular disease has a good response to steroids. In this article we will review relevant literatures of this disorder.