Primary smooth muscle tumours of the thyroid gland are extremely rare neoplasms. There are concerns regarding the management of such disease due to its rarity. Around 22 cases have been reported in the literature till date. In this study, the case of a 63year old female patient who presented with a rapidly enlarging anterior neck mass over the period of 3 months is reported. The patient underwent thyroidectomy with tracheostomy and radiotherapy for the treatment of primary leiomyosarcoma of thyroid gland. Due to similar clinical presentation as anaplastic carcinoma, diagnosis of primary thyroid leiomyosarcomas is difficult and maybe misdiagnosed. Hence the combined assessment of clinical, imaging and pathological data is required.Immunohistochemical analysis is a gold standard for diagnosis. Diagnosis and treatment of the current patient with primary thyroid leiomyosarcomaand a comprehensive review of the relevant literature are presented herein