Vascular Leiomyoma (VL) is a benign soft tissue tumor rarely observed in oral tissues. The aim of this paper is to present a new case of vascular leiomyoma of the lip (VLL), describing its clinical, microscopical, and immunohistochemical features, and review the literature on the tumor. A 27-year-old woman presented with a small painful lesion on lower lip mucosa. Excisional biopsy revealed a well-circumscribed, encapsulated mass formed by small and fusiform cells. Immunohistochemistry analysis revealed intense and diffuse expression of smooth muscle actin (SMA) within the tumor cells and CD34 immunoreactivity of the endothelial cells lining the vascular spaces, indicating the presence of blood vessels. A final diagnosis of VLL was established. No signs of recurrence were observed after five years of follow-up. Although VLL is a rare lesion, it must be considered in the differential diagnosis of nodular lesions on lower and upper lips.