Medullary thyroid cancers account for approximately 7% of primary thyroid malignancies and are often associated with paraneoplastic syndromes. We discuss the case of a 59-year-old man who presented with a hypercoagulable state, nonbacterial endocarditis, and recurrent strokes in the setting of widespread medullary thyroid cancer. The patient was anticoagulated and underwent thyroidectomy, modified radical neck dissection, and subsequent axillary lymphadenectomy. Despite no recurrent thromboembolic events, the patient ultimately succumbed to his disease 8 months after diagnosis and 6 months after initial thyroidectomy. This is the first report, to our knowledge, of this unique presentation in a patient with thyroid cancer. The literature pertaining to the diagnosis and management of hypercoagulability states in patients with cancer, in general, and thyroid cancer, in particular, is reviewed.