Hereby we would like to present a case report of a 27-year-old male patient with drug- -resistant structural epilepsy based on the diagnosis of the polymorphous low‑grade neuroepithelial tumor of the young (PLNTY), first reported by Huse et al in 2017 [1]. PLNTY is a sporadic epileptogenic tumor characterized by an oligodendroglial-like component, diffuse CD34 expression, and alteration of the mitogen-activated protein (MAP) kinase signaling pathway. It shares multiple characteristics with other diffuse low-grade gliomas, especially with oligodendroglioma; nevertheless, its distinction is crucial because of the favorable prognosis of PLNTY.