Low-grade gliomas (LGG) are the most frequent pediatric tumors associated with epilepsy. Molecular sequencing analyses have defined the genomic landscape of these tumors leading to the use of targeted therapies, which have proven to be efficacious. Ongoing clinical trials are testing the incorporation of these drugs, especially in unresectable LGG that progress after standard treatment. Nevertheless, this strategy is not extended in the field of tumor-induced epilepsy. We present a patient with drug-resistant epilepsy secondary to a BRAF V600E-mutated ganglioglioma treated with dabrafenib who showed a dramatic radiological and clinical response with marked improvement in her quality of life.