Abstract
Rhabdomyosarcoma (RMS) is a rare, rapidly growing, highly aggressive malignant neoplasm, originating from undifferentiated mesenchymal cells that retain their ability to differentiate into skeletal muscle. It mainly affects children, accounts for <1% of all adult malignancies and has varied clinical presentations. The head and neck region accounts for 35%-40% of all RMS cases, of which 10%-12% cases involve the oral cavity. This report deals with a case of RMS in a 40-year-old woman, primarily involving maxillary gingiva for which she underwent excision with subsequent recurrences. The uniqueness of this case is that it reminds us of the essential clinical dictum that 'every growth we encounter, no matter how benign it appears clinically, should be looked upon with suspicion'. Hence, proper integration of history, clinical examination and investigation is required to reach a correct diagnosis enabling early treatment, thereby preventing functional and aesthetic loss and psychological trauma.