Background <p>Pediatric central nervous system (CNS) tumors are the most common solid cancers and the leading cause of cancer-related morbidity and mortality in children. The global demographic and epidemiological trends indicate a significant increase in childhood and adolescent cancers, including pediatric brain tumors, in low- and middle-income countries, particularly in Sub-Saharan African countries necessitating the dire need of multidisciplinary Pediatric Neuro-Oncology (PNO) teams to improve outcomes.</p> Objective <p>The primary objective of the study was to evaluate the patterns, clinical presentations, time to diagnosis (TD), and treatment provided to pediatric and adolescent patients with central nervous system tumors who were discussed at the pediatric neuro-oncology tumor board and treated by the neuro-oncology team at Tikur Anbessa Specialized Hospital in Ethiopia.</p> Methods and materials <p>This retrospective cross-sectional study was conducted in the Pediatric Hematology and Oncology (PHO) unit at Tikur Anbessa Specialized Hospital in Ethiopia. It included all pediatric patients under 15 years old with primary central nervous system (CNS) tumors from December 2021 to May 2024. The study aimed to provide an overview of the sociodemographic characteristics of the children, clinical presentation, time to diagnosis, histopathology of the tumors, and treatment modalities recommended by the PNO tumor board.</p> Results <p>A total of two hundred ten patients with pediatric CNS tumors were discussed and reviewed at the Pediatric Neuro-Oncology (PNO) Tumor Board during the study period. More than half of the patients (54.8%, <i>n</i> = 114) were males. The median age at diagnosis was 7 years, and nearly half of the patients (48.6%) were between 5 and 10 years old. The most common clinical presentations were headache (66.2%), vomiting (64.3%), visual symptoms (44.8%), and cerebellar symptoms (43.8%). The median time to diagnosis was 90 days (IQR 60–210), and 60% of the patients presented after three months of symptom onset of the disease. The most common pediatric CNS tumors were medulloblastoma and embryonal CNS tumors, accounting for 32.9% (<i>n</i> = 69), followed by astrocytic tumors; 30.0% (<i>n</i> = 63), craniopharyngiomas (14.0%), and ependymal tumors (11.4%). The main treatments offered by the PNO Tumor Board were a combination of surgery, radiotherapy, and systemic chemotherapy (33.8%), surgery alone (23.8%), and surgery with radiotherapy (21.4%). The PNO Tumor Board was primarily attended by pediatric hematology-oncology fellows, pediatric oncologists (90%), and neurosurgeons (86%).</p> Conclusion <p>This study focused on analyzing the age distribution, clinical presentation, time to diagnosis, burden, and patterns of pediatric primary CNS tumors at the largest tertiary referral center in Ethiopia. It is imperative to prioritize educating healthcare professionals about the symptoms and signs of CNS tumors in children, promoting early diagnosis, facilitating timely referrals, and enhancing the effectiveness of the PNO tumor board. These measures should be considered essential aspects of care for children with CNS tumors.</p>