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 difcult 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.