Tuberculosis (TB) remains a challenge because severe forms occur most frequently in children under 5 years of age and the diagnosis is complex. The objective of this paper was to describe the clinical presentation, frequency, diagnostic methods used and response to treatment in children with TB treated at a tertiary level hospital. The study was retrospective and descriptive of a cohort of consecutive cases treated from January 2010 to December 2013. Ninety-three medical records of children diagnosed with TB according to the definition of the NOM-006-SSA2-2013 were reviewed. Descriptive statistics were used for the analysis. From 93 children, 58% were male (mean age of 7 years), 97% with a history of BCG vaccination, and 6% had contact with a TB case. The most frequent clinical forms were pulmonary (30.1%), lymph node (24.7%), miliary/disseminated (16.1%), meningeal (13%), and osteal TB (7.5%). The most common symptoms were fever and weight loss (50% and 40%, respectively). BAAR and culture were positive in 26% and 7% of all cases, respectively. The histopathological study was conclusive in 90% of the cases. The treatment was successful in 94.6%, with not associated mortality. The association of clinical symptoms with alterations in chest radiography and positive PPD are useful in establishing the presumptive diagnosis and an early and appropriate treatment. Copyright © 2017 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.