Abstract
We report a case of embryonal orbital rhabdomyosarcoma (RMS) in a five year old boy. Immuno-histochemistry of incisional specimen confirmed diagnosis. Eight cycles of chemotherapy along with radiotherapy resulted in over 50% reduction in size of the mass. However, increase in size was noted subsequent to completion of therapy and exenteration was deemed prudent. Margins of the excised specimen were free from tumor cells, but after five months, the patient developed multiple metastases, including skeletal muscle involvement, and died nine months after exenteration, despite repeat chemotherapy along with radiotherapy. Orbital RMS with metastasis to skeletal muscle is a rare entity.