Malignant glomus tumor is a very rare disease originating from the paraganglia system through the body. Glomus tumor, also known as paraganglioma, usually are considered benign, and arises in a variety of head and neck locations, most of which include the carotid body, the vagus nerve, and the jugulotympanic area. The most widely accepted management of benign glomus tumor is surgical extiration. Here, we report a case of recurrent laryngeal glomus tumor which is proven malignant and metastatic to the brain and the lungs. We have treated the patient with combination chemotherapy and radiation to the brain, the result of which is partial response in terms of decreased size of metastatic lung lesions.