OBJECTIVE: Brain tumors are the second most common malignancy in children and the most common cause of cancer related deaths. Major advances in management in terms of surgery, radiation and chemotherapy have led to better outcomes. Delayed diagnosis, advanced disease at presentation, late referrals, nosocomial infections, delays to radiotherapy and poor support services are the major reasons for poorer outcomes in developing countries. Little is known about the profile of brain tumors in Pakistan. This study aims to describe the clinical profile and outcome of primary brain tumors in children at a single tertiary center in Pakistan. METHODS/MATERIALS: All children (0 – 16 years) with primary CNS tumors from 2004 till 2014 at Aga Khan University Hospital were reviewed retrospectively for clinical data, demographics, radiological findings, management and outcome. RESULTS: 175 children were included in the study. Male to female ratio was 1.4:1. Most of the patients were in 5 -10 years age group (38.9%). Most common presenting complains were headache 115 (65.7%) & vomiting 100 (57.1%). Predominant site was supratentorial 91 (52%). Three patients (1.7%) had family history of cancers. Glial tumors were 93 (53.1%) followed by embryonal 40(22.9%), Craniopharyngiomas 25 (14.3%) & Germ cell 1 (0.6%). Low grade astrocytoma (23.4%) were the most common glial tumors while Medulloblastoma (15.4%) was the most common embryonal tumor. Majority of the patients underwent surgery only (53.7%). Radiation was given to 48 (27.4%) patients. Half of the patients 79 (45.1%) were lost to follow up. Patients expired were 41 (23.4%), 24 (13.7%) are alive with residual disease while 22 patients (12.6%) were cured. CONCLUSION: This is the only study from Pakistan showing demographics of the children with brain tumors. Significant improvement needs to be made for timely diagnosis, early referrals and collaborated team efforts with multidisciplinary tumor board to improve outcome.