Extraventricular neurocytoma (EVN) was classified as a World Health Organization (WHO) grade II tumor; however, EVN is not fully understood; it presents a variable histological feature that included oligodendroglioma-like, neuropil-like matrix, ganglion or gangloid cells, perivascular pseudorosettes, vessel hyalinization, calcifications, and myxoid degeneration. In some very rare cases, atypical histological features such as increased mitotic figures, focal necrosis, endothelial cell proliferation, and Ki-67 index of >2% made this tumor more aggressive and more susceptible to recur. We present the case of a young patient who presents with a 2-year history of seizure without other symptoms. Magnetic resonance imaging reveals a parietal lobe and well-circumscribed lesion treated by gross total resection and adjuvant radiotherapy. Clear guidelines to treat this kind of lesions are not well established and there is not a consensus of correct treatment in these tumors.