BACKGROUND: Uterine leiomyosarcoma is an aggressive tumor that has a propensity for distant metastasis. Distant sites of spread include the lung, liver, brain, and bone. CASE: A woman in her 5th decade was diagnosed with stage IV leiomyosarcoma of the uterus. Distant metastasis in the chest was confirmed by thoracotomy at the time of her original diagnosis. She subsequently experienced multiple episodes of metastasis including metastatic disease to her thyroid. She survived 71 months after diagnosis of stage IV disease. CONCLUSION: Our case represents the first reported experience with uterine leiomyosarcoma metastatic to the thyroid. Additionally the case is notable because the patient experienced multiple episodes of asymptomatic stable disease after repetitive chemotherapeutic regimens.