Extrapulmonary tuberculosis is not a rare infection of children in Taiwan. From 1985 to 1989, we studied 39 children with the diagnosis of extrapulmonary tuberculosis from a total of 43 sites. They were diagnosed by routine diagnostic tests including bacteriologic cultures in 11, pathological features in 25, and clinical presentations in 2 patients. Positive mycobacterial DNA probe method was obtained from a patient with tuberculous meningitis. The most common sites of involvement were lymph nodes in 9 (20.9%), meninges in 7 (16.3%), bone in 7 (16.3%), joint in 6 (14.0%) and miliary infection in 6 (14.0%). The median age of the 2 patients with renal tuberculosis was 14.5 years, which was older compared to the patients with other organ systems involvement. Tuberculin skin tests were significantly positive (> 10 mm) in 54% of the tested children. Among the treated patients, 76% were cured after 9 to 12 months of antituberculous chemotherapy without sequelae, while 24% had sequelae associated with tuberculous infection despite treatment. There was no death reported during the study period. We conclude that extrapulmonary tuberculosis remains an important health problem to the pediatric population of this island despite the overall decline in the incidence of tuberculosis. Early detection and thorough treatment for a suitable period are mandatory to improve the prognosis of this potentially curable infectious disease.