Symplastic glomus tumors are defined as glomus tumors with a high-grade nuclear pleomorphism in the absence of any other malignant features, such as large size, deep location, infiltrative growth, mitotic activity, or necrosis. Only 11 cases have been reported so far in the English literature. It could be a challenge for pathologists who have no experience with it because the tumor can show marked nuclear atypia and pleomorphism. Despite its high nuclear grade, a symplastic glomus tumor itself has a benign biologic behavior. Hereby, the authors report a case of a symplastic glomus tumor on the right index finger tip of a 44-year-old woman with a literature review.