Rhabdomyosarcoma (RMS) is a rare, aggressive, malignant mesenchymal tumor of skeletal muscle
cells. The pleomorphic histological variant of RMS occurs in adults beyond 45years of age and represents
the most aggressive subtype with an incidence of approximately 0.44/100,000. The diagnosis of RMS is difcult with the 5year
overall survival rate less than 50%. It presents varied clinical and biological behavior and requires individualized
management. The common region of metastasis includes lymph nodes, lungs and bone marrow. Here we report a case of
pleomorphic rhabdomyosarcoma in 58year old female. The patient reported with a swelling in the neck region and had a history
of surgical treatment for ovarian tumor. Histopathology ndings revealed metastatic undifferentiated carcinoma. The case was
positive for immunohistochemistry markers and their ndings are diagnosed as pleomorphic rhabdomyosarcoma. This is a rare
case of RMS which had metastasized to head and neck and this article emphasizes the importance of IHC in accurate and clear
diagnosis of RMS.