Extrapulmonary involvement can occur in isolation or along with a pulmonary focus as in the case of patients with disseminated tuberculosis (TB). The recent human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) pandemic has resulted in changing epidemiology and has once again brought extrapulmonary tuberculosis (EPTB) into focus. EPTB constitutes about 15 to 20 per cent of all cases of tuberculosis in immunocompetent patients and accounts for more than 50 per cent of the cases in HIV-positive individuals. Lymph nodes are the most common site of involvement followed by pleural effusion and virtually every site of the body can be affected. Since the clinical presentation of EPTB is atypical, tissue samples for the confirmation of diagnostic can sometimes be difficult to procure, and the conventional diagnostic methods have a poor yield, the diagnosis is often delayed. Availability of computerised tomographic scan, magnetic resonance imaging laparoscopy, endoscopy have tremendously helped in anatomical localisation of EPTB. The disease usually responds to standard antituberculosis drug treatment. Biopsy and/or surgery is required to procure tissue samples for diagnosis and for managing complications. Further research is required for evolving the most suitable treatment regimens, optimal duration of treatment and safety when used with highly active antiretroviral treatment (HAART).