Burkitt lymphoma is a high-grade and aggressive malignant lymphoma classified as a mature B-cell neoplasm. Although the disease is generally carries a poor prognosis, some cases are curable. Its rapid growth and trend toward multifocal and often extranodal spread including the central nervous system are the main causes of its poor prognosis. We describe a case of Burkitt lymphoma arising in the oral region that was detected because of numbness of the lower lip. A 28-year-old woman felt numbness of the lower lip and swelling of the lower gingiva. She was admitted to our hospital for neurological examination, and a biopsy of the right lower gingiva revealed Burkitt lymphoma. The patient was transferred to hematology ward. Wholebody positron-emission tomography and computed tomography showed mass shadows in the mandible, breasts, abdomen, pelvis, and femoral bone. Therefore, the disease was evaluated to be stage IV according to the Murphy staging system. Multicycle chemotherapy including R-hyper CVAD and preventive intrathecal treatment finally resulted in disappearance of the gingival swelling and other tumors. Clinical symptoms also resolved. Clinical remission has been maintained for 4 years.