Objective To evaluate the clinical features and treatment outcomes of acute retinal necrosis syndrome (ARNS) which caused by long-term usage of immunosuppressent drug. Methods The clinical data of 8 patients (12 eyes) with immunocompromised-related ARNS were retrospectively reviewed. The diagosis was made by medical history, slit-lamp microscopy, pre-set lenses check, serologic examination and fluorescein fundus angiography. The patients (2 males and 6 females) aged from 35 to 54 years, with the mean age of 44.2 years. Previous medical history included hematopathy (2 cases), thymus tumor (2 cases, one also with meningoencephalitis), meningoencephalitis (2 cases), systemic lupus erythematosus (1 case) and acute pneumonia (1 case). All patients received immunosuppressent therapy for a long time before ARNS occurred. The visual acuity was ＜0.05 (5 eyes, 41.7%), or 0.05-0.3 (3 eyes, 25.0%), or 0.3-1.0 (4 eyes, 33.3%). Those ARNS patients received antiviral therapy, laser photocoagulation and (or) surgery therapy. The mean followed-up period was 10.8 months (from 3 to 36 months). Results The eye sympotoms and uveitis of all patients were very mild, and their retinal vasculitis and retinal necrosis progressed slowly. Retinal vasculitis was involved in 4 quadrants (7 eyes), or 1-2 quadrants (2 eyes), or ＜1quadrant (3 eyes). Retinal necrosis extended from peripheral retina to mid- peripheral retina (10 eyes, 83.4%), or from peripheral retina to posterior pole (2 eyes, 16.6%). At the end of the follow-up period, the visual acuity of 7 eyes (58.3%) showed different degree of improvement. The follow-up visual acuity was ＜0.05 (4 eyes, 33.3%), or 0.05-0.3 (2 eyes, 16.6%), or 0.3-1.0 (6 eyes, 50%). Conclusions Immunocompromised ARNS patients had valid medical history and typical clinical features. However the eye sympotoms and uveitis were very mild, retinal vasculitis and retinal necrosis progressed slowly in this study. Early diagnosis and prompt therapy may save the visual acuity of those patients.