This case demonstrated malignant transformation of PXA into a high-grade neuroepithelial tumor, notably progressing to a neoplasm featuring primitive neuroepithelial differentiation, focal spindle cell sarcomatous components, and characteristic genetic alterations—including BRAF V600E mutation and CDKN2A homozygous deletion. Here, BRAF is linked to pleomorphic xanthoastrocytoma.