Embryonal rhabdomyosarcoma is an exceedingly rare tumor in adult. We report an embryonal rhabdomyosarcoma of the ethmoid in a 59-year-old Iban lady who presented with proptosis and complete ptosis of her left eye for two months. Imaging investigations showed left ethmoidal and left orbital soft tissue mass with extradura and dura involvement. The patient was planned for chemotherapy. Unfortunately, in such an advanced disease, she succumbed before treatment.
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