Thyroid sarcoma is a very rare entity, accounting for less than 1% of all malignant thyroid tumours. Rhabdomyosarcoma (RMS) is a sarcoma subtype, which is more common in children and adolescents. In this case, a 68-year old man, presented with hoarseness and diagnosed with pleomorphic RMS, was explored. No study of primary thyroid pure RMS has been reported in the literature, with the exception of the case reports of differentiated RMS.