Primitive neuroectodermal tumor involving the frontal skull base in an infant

We report here a case of a primitive neuroectodermal tumor involving the frontal skull base in a 5-month-old male infant. He presented with rapidly progressing exophthalmos on the left side. Preoperative magnetic resonance imaging revealed a well-demarcated, enhanced mass of 6 cm in diameter in the intradural extra-axial frontal region. The tumor was totally removed by surgery. Pathological findings were consistent with a primitive neuroectodermal tumor (PNET). Intradural extra-axial PNET is very rare, and its prognosis is poor.