BACKGROUND: Carcinoma cuniculatum, a well-differentiated squamous cell carcinoma, is a rare neoplasm with a low risk of metastasis. METHODS AND RESULTS: A 74-year-old female patient is presented with a carcinoma cuniculatum of the right maxilla, a very rare variant of squamous cell carcinoma that usually occurs in the skin of the lower extremities, in particular, in the skin of the plantar surface of foot. It is described by an invasive growth pattern, but metastases to regional lymph nodes are rare. In the oral cavity, only very few cases have been published. CONCLUSION: The correct diagnosis of this entity with the knowledge that it is a variant of a low-grade carcinoma with low risk of metastasis is important, so that elective neck dissection must not be obligate.