Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and 
are composed of small round cells. Because of their rare occurrence, optimal 
therapy is challenging, particularly if they occur in the head and neck. 
Diagnosis is based on history, immunostaining with at least 2 neural markers, 
ultrastructural examination, and evidence of an abnormal t(11;22)(q24;q12) 
translocation as the hallmark for the Ewing's sarcoma family. The prognosis in 
general is poor because of overt metastasis at the time of diagnosis. Of 27 
reported patients with primitive neuroectodermal tumors of the head and neck, 23 
were less than 20 years of age. Most patients presented with a tumor in the 
nasal cavity, paranasal sinuses, or neck. Symptoms developed rapidly (3.6 
months, on average), and a lethal outcome occurred in 9 patients. This highly 
malignant tumor requires an aggressive combination of radical resection, 
chemotherapy, and radiotherapy. A close follow-up with regular radiographic 
examination for at least 5 years is mandatory.