Objective: To explore the status quo and development suggestions of the availability of medical insurance for orphan drugs in China through the analysis of international, national and local rare disease drug use system and policy, and the species of orphan drugs in China’’s health insurance directory. Methods: The website of local government and the Chinese database were retrieved by computer, and the relevant policies and regulations of rare disease and orphan drugs were obtained. The information of rare diseases and orphan drugs in “national basic medical insurance, employment injury insurance and maternity insurance drug Catalog” was systematically extracted. Results: There is no official definition of rare diseases and the official list of rare diseases in China. In recent years, with regard to the approval policy and measures of rare diseases and the R & D of orphan drugs are increasingly full. In the medical security, many provinces and municipalities have been introduced for the popular characteristics of local rare diseases and the implementation of the rare diseases protection policy, however, the whole policy of nation level is still blank. The edition of 2017 of the basic medical insurance, industrial injury insurance and maternity insurance drugs directory contains 53 kinds of orphan drugs, added 7 kinds compared to the version of 2009 version, an increase of 15.22%. 17 kinds of rare diseases were covered, involving blood diseases, bone or cartilage diseases, congenital metabolic disorders, brain or nervous system diseases, immune system diseases, skin diseases, digestive system diseases, endocrine system diseases and other treatment fields. Compared with previous years, the new version of the essential insurance medicine list of orphan drugs also increased the dosage form, which class A has more types of formulations rather than class B. Conclusion: China’’s rare disease social security system policy focused on the field of R & D approval, medical security is dominated by local policy-based, the scope of orphan drugs in the new edition of national health insurance drug list is expanded, but the supporting policies need to be improved. Therefore, the problem of rare disease social security system policy in China needs to be paid more attention.