Abstract Rhabdomyosarcoma occurs commonly in the head and neck, seen mostly in the parameningeal region. We report, a 5-year-old boy presented with a history of painless progressive protrusion of the left eye (LE) and progressive headache and strabismus suddenly with the impression of rhabdomyosarcoma of mastoid protruded to orbit, who resistant to conventional and salvage chemotherapy. Finally, he was cured by metronomic chemotherapy (Vinorelbin and Cyclophosphamide) completely