Objective: To report the effect of pyrimethamine treatment in ocular toxoplasmosis which failed to treat with baktar and clindamycin in a 43-year-old male with acquired immune deficiency syndrome (AIDS). Methods: Retrospective interventional case report. Results: This case presented with left blurred vision, and left fundoscopy revealed necrotizing retinitis, frosted angitis and kyrielesis plaques. These findings were characterized with toxoplasmosis uveitis with elevated aqueous toxoplasmosis IgG level. He was undergoing HARRT, anti-tuberculosis therapy combined with trimethoprim-sulfamethoxazole (baktar) and clindamycin for systemic opportunistic infection, but toxoplasmosis uveitis still occurred. We started full pyrimethamine therapy for 28 days and the retinitis resolved gradually without recurrence at 14 months follow-up. Conclusions: Pyrimethamine treatment rather than baktar and clindamycin therapy is effective for AIDS-related toxoplasmosis uveitis.