The peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant soft tissue neoplasm in children and young adults. Fewer than 10 reported cases of pPNET of maxilla are available in the English literature. A 28-year-old woman was presented with the pPNET of the maxilla and metastasis. Two years after diagnosis, she experienced diplopia, and then magnetic resonance imaging w...