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Abstract Background Central nervous system (CNS) tumors remain a significant cause of morbidity and mortality in children. Designing cancer control strategies requires adequate data, which is scanty in the sub-Saharan Africa. We describe the demographics, histopathology, management and outcome after tumour surgery at Cure Children’s Hospital (CCHU), a specialized paediatric neurosurgical hospital in Uganda. Methods In this retrospective cohort analysis of all children under 18 years who had CNS tumour surgery at CCHU in 2024, we collected demographic and clinical data. Fischer’s exact test was used to assess the risk factors for mortality. Results Eighty-nine patients had surgery of whom 47(52.8%) were male and 42(47.2%) were female. All had brain tumours except one with a spinal tumour. Median age at diagnosis was 5.0(IQR 0.5-17) years with 37.1% under 5 years and 33.7% between 5-10 years of age. The commonest histopathological types were ependymoma (15.7%), medulloblastoma (15.7%) and craniopharyngioma (10.1%). Supratentorial tumours (51.7%) were most common. Postoperative complications occurred in 37(41.6%) patients and cerebrospinal fluid leak was the commonest in 20(54.1%). Median number of surgeries per child was 3(IQR 2-9) for those who developed post operative complications and 1(IQR 1-4) for those who did not. Eighty-five (95.5%) patients were referred for adjuvant therapy at Uganda Cancer Institute (UCI). Only 55(64.7%) reached UCI of whom 29 (52.7%) received adjuvant therapy. 60(67.4%) are alive, 21(23.6%) are dead, and 8(9.0%) are lost to follow up. Majority 14(66.7%) died at home after surgery but before accessing adjuvant therapy. Failure to access adjuvant therapy was significantly associated with mortality (p < 0.005). Conclusions Failure to access adjuvant therapy reduces the chances of survival even after successful tumour surgery. Collaboration between surgeons and oncologists may improve access to adjuvant therapy and subsequently improve survival.