Abstract: Pleomorphic xanthoastrocytoma (PXA) is a primary central nervous system tumor characterized primarily by epileptic seizures as its clinical manifestation. Due to overlapping clinical and radiological findings with other intracranial tumors, and its rarity in clinical settings, PXA is easily misdiagnosed. Here, we report a case of PXA in a 16-year-old male who experienced a sudden epileptic seizure. Cranial magnetic resonance imaging (MRI) showed a cystic-solid lesion in the left temporal lobe, which was interpreted as a possible glioma. The patient underwent a gross total resection of the tumor, and the pathological examination confirmed the diagnosis of PXA (World Health Organization grade 2). After the previous treatment, the patient did not experience any epileptic seizures. During a follow-up period of approximately 1 year, a cranial MRI indicated no signs of tumor recurrence. In this report, a comprehensive literature review based on the imaging findings in this case was conducted to summarize the imaging features of PXA, aiming to enhance the clinicians’ understanding and provide guidance for accurate diagnosis.