A 57-year-old female patient presented to the Dentistry Department with tumor on the upper lip. A macroscopic examination revealed a well-circumscribed, smooth, firm, round nodule with a diameter of 0.5 cm (Fig. 1). Histologically, it was composed of anastomosing duct-like or trabecular structures lined by a single or double layer of columnar cells (Fig. 2), which were immunoreactive to cytokeratin CK7 (Fig. 3), CD117 (Fig. 4) and S100 protein (Fig. 5). The histological texture and immunohistochemical characteristics of primary tumor, localized in the neighbourhood, excised 2 years earlier were similar to the microscopic picture of the presented neoplasm.