A glomus tumor is a benign neoplasm originating from the modified smooth muscle cells of the vascular glomus. This neoplasm was firstly considered by Masson in 1924, and generally it has a solitary lesions but in some cases multiple lesions were described. Differential diagnosis is usually made with Kaposi sarcoma and hemangiopericytoma. The histopathologic examination is essential together with the immunohistochemical study. The clinical case of a 65-year-old man with a glomus tumor of the lower lip is described.