Dysembryoplastic neuroepithelial tumors (DNTs) are benign brain tumors classified as grade 1 in the 2021 World Health Organization (WHO) classification of central nervous system tumors. DNTs rarely undergo malignant transformation and cause symptomatic intracranial hemorrhage. We report a case of malignant transformation of DNT presenting with intraventricular hemorrhage and review the literature on malignant transformation of DNTs. An 18-year-old woman with a history of epilepsy presented with a sudden headache and vomiting. Radiological examination revealed a mass lesion in the left parietal lobe and intraventricular hemorrhage. The patient underwent an emergency craniotomy for brain tumor resection. The lesion was pathologically diagnosed as a malignant transformation of DNT. She had been followed up without tumor recurrence for 2 years after surgery.