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Carcinoma cuniculatum (CC) is a rare variant of squamous cell carcinoma. We describe the clinicopathologic findings in a new case.This is a literature review and retrospective study of a case with CC.The clinical and imaging findings of CC are diverse; some cases might be challenging to diagnose accurately by biopsy. This article reports a case of CC that occurred in the retromolar region with involvement of the mandible. The patient was a 39-year-old man. Clinically, the lesion manifested as an ulcerative mass in the mandibular molar region. Imaging results showed that the lesion in the jaw exhibited moth-eaten destruction without obvious expansion of the jaw bone. The postoperative pathologic examinations were consistent with CC; additionally, metastases were present in 1 level II lymph node and 1 submandibular lymph node. Left soft tissue metastasis and right cervical lymph node metastases were detected 10 and 27 months after surgery, respectively.CC is a rare epithelial malignant tumor and has variable clinical manifestations. The diagnosis of CC mainly depends on pathologic features. Most patients with CC have a good prognosis; only a small percentage of patients might experience lymph node metastasis.