IgG4-RD is a newly recognized chronic and systemic disease. IgG4-positive plasma cells play a part in the occurrence and development of this disease. “IgG4-related ophthalmic disease(ROD)” was proposed for the involvement of eyes in general. The clinical manifestations of IgG4-ROD are various due to different involved parts and have unique pathologic features compared with other organs. With a better understanding on this disease in recent years, diagnosis and treatment rate has been improved. In this paper, we will discuss the definition, epidemiology, pathogenesis, clinicopathological features, diagnosis and differential diagnosis, serum biochemical indices, imaging examinations and treatment about IgG4-ROD.