Introduction: this paper will report a case of carcinoma cuniculatum (CC) in maxillary gingiva along with a literature review related to the research topic which specifically focused on cases occurring exclusively in the oral cavity.

Observation: A 44-year-old man presented with an exophytic mass in maxillary alveolar region characterized by slow growth and the presence of bone erosion. The diagnosis of CC was established on the resected specimen. After surgery, the patient underwent adjunct radiotherapy and chemotherapy.

Discussion: Carcinoma cuniculatum (CC) is a rare variant of squamous cell carcinoma (SCC). Oral carcinoma cuniculatum is even rarer and is often initially misdiagnosed; it presents clinically as an exophytic growth and slowly penetrates the jaw, causing deterioration of the underlying bone. The primary treatment for carcinoma cuniculatum involves surgical resection.

Conclusion: this case report highlight the challenges involved in arriving at such an uncommon diagnosis while also discussing the diagnosis of CC based on the combination of clinical symptoms and the pathological findings.