Oral angiokeratoma is a rare vascular lesion that has various clinical presentations. It usually occurs as part of generalized angiokeratoma and rarely appears as a solitary lesion with no underlying systemic diseases. Only 33 cases were reported so far worldwide. In this case report, we present a rare case of isolated solitary oral angiokeratoma in a 22-year-old female patient, which is the first case to be reported in the labial mucosa that has been treated successfully by surgical excision.