Opportunistic infections of the central nervous system (CNS) are very common and severe complications of advanced immunodeficiency in patients with human immunodeficiency virus type 1 (HIV-1) infection, which are included in the diagnostic criteria for acquired immunodeficiency syndrome (AIDS) defining conditions according to 1993 Revised Classification System for HIV Infection and Expanded Surveillance Case Definition for AIDS among Adolescents and Adults published by USA Centers for Disease Control and Prevention (CDC). The etiologic microorganisms of CNS opportunistic infections include virus, bacteria, fungus, mycobacterium and parasite. The clinical symptoms, signs and laboratory examinations of these diseases are different from that of patients with non-immunodeficiency. Even in the era of highly active antiretroviral therapy (HAART), worsening conditions or new infections may occur. Therefore, prompt diagnosis and treatment of such disorders are critical. The immune reconstitution inflammatory syndrome (IRIS) in HIV-1 infected patients in the initiating antiretroviral therapy results from restored immunity to specific infectious or non-infectious antigens. This study reviews the epidemiology, pathogenesis, clinical features, diagnosis of some common CNS disorders in HIV-1 infected patients. Physicians caring for such patients must be aware of the new diagnostic modalities and therapeutic options of these diseases.