Background Primary thyroid leiomyosarcoma(LMS) is very rare, with only 30 cases reported worldwide. Thyroid LMS should be diagnosed with caution and comprehensively evaluated considering clinical, imaging, and pathological data. It is a kind of fatal tumor with an extremely poor prognosis and lacks effective treatment regimens, and most patients die within 1 year of diagnosis. Here, we present a case report on a patient with Primary thyroid LMS. Case presentation A 76-year-old woman presented with hoarseness, dysphagia, and dyspnea when lying down for the past 1 month visited our hospital. She had a history of right neck masses in the past 2 years. A hard, irregular tumor of approximately 8 cm × 5 cm × 5 cm was observed on the right lobe of the thyroid, the boundary between which and the surrounding tissues was unclear. The tumor anteriorly invaded the cervical muscles and interiorly invaded the right walls of the trachea and esophagus. Most tumor tissues were excised, a curative operation seemed impossible. Immunohistochemically, the tumor cells were positive for Calponin, vimentin and SMA, but negative for EMA, CK, P53, Desmin and S-100. Tumor recurrence and progression were considered two months after surgery, the patient refused follow-up treatment for personal reasons and died 4 months after surgery. Conclusion Thyroid LMS accounting for merely 0.014% of primary thyroid tumors of unknown etiology. The diagnosis can only be confirmed upon a lack of evidence regarding epithelial differentiation or other types of sarcoma differentiation and when immunohistochemistry yields positive smooth muscle markers. Primary thyroid LMS is primarily surgically resected and no other effective treatment currently available. Disease progression is rapid, the prognosis is poor, and the 1-year survival rate is <10%. In the present study, a rare case of a 76-year-old female patient diagnosed with thyroid LMS was reported and a review of the literature is presented.