Background: We encountered a rare case of central giant cell granuloma(CGCG)in association with neurofibromatosis type 1 in a middle-aged Asian woman. Most reported cases involve isolated central giant cell granuloma or neurofibromatosis type 1(NF1), and concurrence of these two entities is very rare.Methods: We report a case of concurrent central giant cell granuloma with neurofibromatosis type 1. Thorough clinical and radiological examinations were performed.Result: After diagnosis of possible concurrent central giant cell granuloma with neurofibromatosis, surgical excision with curettage was performed. Based on histopathological and clinico-radiological findings, the final diagnosis was central giant cell granuloma with neurofibromatosis 1. Follow-up at 1 year did not show any recurrence.Conclusion: We review the proposed mechanisms underlying the apparent association between CGCG of the jaws and neurofibromatosis 1. This case could represent either a coincidental association or a true genetic linkage; the mechanism in the present case appears most likely related to NF1-related osseous dysplasia.