Primitive neuroectodermal tumor (PNET) of the orbit is rare. We present a case of a young patient with orbital PNET extending to the endo-crane and sinuses, which had been treated 10 years before with the diagnosis of rhabdomyosarcoma. The diagnosis was corrected with immunohistochemistry. Through this observation, we discuss the clinical aspects, the diagnosis difficulties, and the therapeutic options of this rare affection.