OBJECTIVE: To determine factors related to survival in acquired immune-deficiency syndrome (AIDS) patients with extra-pulmonary tuberculosis (EPTB), when this condition is the first AIDS-defining disease. DESIGN: A retrospective cohort-study of 549 AIDS patients with EPTB as the first AIDS-defining disease. Potential candidates to predict survival were sex, human immunodeficiency virus (HIV) exposure, the coexistence of pulmonary and EPTB at diagnosis, tuberculin skin test, directly observed therapy for tuberculosis (DOT), and highly active anti-retroviral therapy (HAART). The Kaplan-Meier method and Cox regression models were used to assess factors associated with survival. RESULTS: Estimated 3-year survival was 47.0% for those diagnosed before 1993, 72.6% for patients with first AIDS diagnosis during 1995-1996 and 84.6% for those diagnosed after 1996. A negative tuberculin test (hazard ratio [HR] = 1.6, 95% CI 1.1-2.3), not being on DOT (HR 2.2; 95% CI 1.3-3.7) and having pulmonary tuberculosis involvement also (HR 1.3; 95% CI 1.1-1.7) were independently associated with poorer survival. The survival of patients significantly improved after the introduction of HAART (HR 0.4; 95% CI 0.2-0.6). CONCLUSION: The survival of HIV patients with EPTB as their first AIDS-defining disease has substantially improved during the last decade. A negative tuberculin skin test and not receiving DOT are associated with poorer survival among HIV-infected patients whose first AIDS-defining disease is EPTB.