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Background Rosette-forming glioneuronal tumors (RGNT) are slow-growing WHO Grade I tumors that are characterized by mixed histology and rosette formation. Although typically located in the posterior fossa, these tumors can rarely originate elsewhere. Here, we describe the fourth case in literature where an RGNT was localized to the lateral ventricles and detail the treatment approach. Case description A 41-year-old male presented with a 10 day history of gradually worsening headaches and mild gait difficulty. Computed tomography and ma