Introduction: Neuroblastoma, also known as neural crest tumor, is the most common cancer in children under 5 years old. Approximately 1 to 2% of cases are hereditary. It predominantly affects the adrenal gland and rarely manifests as a primary cancer of the central nervous system.

Patients and methods: This concerns a 3-year-old patient who underwent surgery at the neurosurgery department of the National Institute of Neurology for an intraventricular neuroblastoma.

Case report: We report the case of a 3-year-old child with a family history of brain tumors, who presented with symptoms of intracranial hypertension associated with gait disturbances. Neurological examination revealed cognitive decline with ataxic gait deviated to the left. The rest of the examination was unremarkable. Brain MRI showed a lesion at the ventricular junction extending into the right lateral ventricle, lobulated and fleshy with multiple calcifications. The patient underwent surgery: macroscopically complete resection via a transcortical approach of a highly hemorrhagic and friable heterogeneous lesion, with placement of an external ventricular drainage valve. Postoperative course was complicated by meningitis, which was managed with antibiotic therapy. Histopathological examination concluded a diagnosis of neuroblastoma. The patient received adjuvant chemotherapy.

Conclusion: Neuroblastoma is a malignant tumor arising from neural crest cells, which typically develops in the adrenal glands or the sympathetic nervous system. Intracranial localization is rare and can either be metastatic or, more rarely, primary. Neuroblastoma is generally associated with numerous genetic abnormalities. The diagnosis of primary neuroblastoma relies primarily on histopathological examination. Therapeutic approaches include surgical resection followed by chemotherapy. Prognosis mainly depends on the extent of resection, as neuroblastoma is sensitive to chemotherapy but not curable by it.