Primitive neuroectodermal tumor of the maxilla

Primitive neuroectodermal tumor (PNET) of the maxilla is a soft tissue sarcoma classically described under small round blue cell tumors. This tumor is commonly seen in the chest, pelvis, abdomen, and extremities; and rarely in dura, ovaries, cervix, etc. These tumors are rare in the head and neck area. We report a case of a 3-year-old girl presenting with a PNET of the maxilla.