Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing, benign tumor originating from the neural crest. The tumor most often occurs during the first year of life. The predilection site of MNTI is the anterior maxilla, whereas lesions of the mandible are uncommon and account for only 6% of all cases. At present, the most common treatment for MNTI is surgical resection, however, tumor recurrence arises in 10-60% of cases. The optimal extent of surgical resection is a matter of debate; rapid growth and the possibilities of malignant transformation and metastasis indicate aggressive surgical resection. However, extensive resection may interfere with post-operative growth and development. The procedure should therefore preserve as much of the surrounding tissue as possible. The present case study examines a rare case of right-sided mandibular MNTI in a two-month-old female. The association between the tumor and the surrounding sclerotin, affected dental germ and the condition of the inferior alveolar nerve, were observed during the operative and post-operative periods. In addition, previous cases of MNTI were reviewed to evaluate the optimal scope of surgical resection.