Background: We studied the clinical presentation, diagnosis, treatment outcome and survival in children with HIV and Tuberculosis (TB) co-infection. Methods: All HIV infected children with symptoms or signs consistent with tuberculosis were screened. We studied 24 cases of culture confirmed TB from among a cohort of 213 HIV infected children. All these children were on HAART for their HIV infection. Information on TB was collected by retrospective chart review of these children. Results: In a cohort of 213 children with vertically transmitted HIV infection, a total of 76 (36%) children suspected to have tuberculosis based on their clinical presentation together with either a positive Mantoux test or AFB positivity and treated for TB. Twenty four children had culture positive TB. The median age at diagnosis of TB was 16 months. Over half of these children had some immunodeficiency. Common presentations were fever (87%), history of contact with an open case of TB (79%), cough for more than 2 weeks (75%), malnutrition (71%), hepatosplenomegaly (71%), chronic diarrhea (67%) and generalized lymphadenopathy (58%). Mantoux test result was positive in 12 (50%) patients. Chest roentgenograms were abnormal in all the children, with hilar and/or Para tracheal node (62%) and lobar or segmental opacification (57%). Twenty one (87%) children had pulmonary TB at the time of their diagnosis. One or more sites of Extrapulmonary TB were confirmed in 10 (41%) patients. After six months of ATT, the cure rate was 64%. Three patients had documented drug-resistant. Five children (20%) died. Conclusion: TB is a common co-infection in HIV infected children and children often present with un-resolving pneumonia. It carries significant mortality despite the HAART and adequate anti-tuberculosis treatment in these children.