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Introduction: Tuberculosis (TB) remains a major global health problem affecting millions of people annually. Tuberculosis in children has unique features different from adults which makes the diagnosis to be more difficult. The spectrum of the symptoms of TB in children could vary from non-specific symptoms to severe clinical presentations. In this study, we reviewed our experience at pediatric patients with active TB admitted in a tertiary hospital and aimed to compare the epide­miological, clinical and microbiological features of children with the extra-pulmonary tuberculosis(EPTB) and pulmonary tuberculosis(PTB). Material and methods: Patients under 14 years of age diagnosed with active TB in our unit between December 2008 and September 2013 were included in the study. Data including demographic characteristics, clinical history, microbiology, imaging studies, medications and outcomes of the patients were collected from medical records. Results: A total of 129 cases of active TB were identified. Ninety-two (78.6%) of the cases had Pulmonary Tuberculosis and 25 (21.4%) of the cases had Extra-pulmonary tuberculosis. The most common signs and symptoms on admission were fever in 40 cases (34.2%) and cough in 81 cases (69.2%). The number of patients without symptoms including fever, cough, malaise and weight loss were significantly higher in EXPTB (72.0%) group when compared with patients in PTB group (13.0%) (p 0.05). The detection rate of source in PTB group (42.4%) was significantly higher than the rate in EPTB group (20.0%)( p=0.04). In the drug-resistant group, no source of infection could be established in 5 of 9 patients (55.5%). Conclusions: Extra-pulmonary tuberculosis diagnosis is more difficult than Pulmonary tuberculosis in children due to the various problems such as absence of associated pulmonary involvement, lack of constitutional symptoms and negative tuberculosis exposure history compared to Pulmonary Tuberculosis. New strategies are required for improving the diagnosis of Extra-pulmonary tuberculosis in children