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Background:   Ganglioglioma (GG) of the third ventricle is rare. Surgical excision of tumors in this location is associated with high morbidity due to nearby eloquent brain centers. Alternative treatments, when available, should be considered to reduce risks of surgical treatment.    Case Description:   We present the case of a 21-year-old female diagnosed with a BRAF V600E mutated GG of the third ventricle. After an endoscopic biopsy and insertion of a ventriculoperitoneal shunt, the patient was started on the  BRAF  inhibitor dabrafenib, as an alternative to surgery or radiation. Nearly 2 years after starting dabrafenib, her tumor appearance on serial magnetic resonance imaging is stable, and she has maintained a good quality of life with no new neurological symptoms.    Conclusion:   The disease control thus far suggests targeted medical therapy of GG of the third ventricle with  BRAF  inhibitors may have efficacy and should be a considered treatment modality.

Treatment of BRAF V600E mutated ganglioglioma of the third ventricle with dabrafenib