婴儿黑色素神经外胚瘤（MNTI）是一种少见的、增长迅速的神经脊来源良性肿瘤，多发生于1 岁以内婴幼儿。 好发部位为上颌骨前部，下颌骨的病例少见。 本文报道1 例发生于下颌骨并行手术治疗的MNTI 病例，并通过文献回顾探讨MNTI 的病例特点及诊断治疗要点。 口腔婴儿MNTI 具有典型的发病部位与发病年龄，临床检查时应注意肿物的颜色。 肿瘤常生长迅速，具有局部侵袭性。 影像学检查表现为边界清楚的透射影， 典型的CT 影像为边缘不规则的低密度团块，偶有高密度的报道。 MRI 的T1 加权和T2 加权上均表现为低信号团。 病理检查常由大的并含不等量色素颗粒的上皮样细胞和小的神经母细胞样细胞组成。 目前常用的治疗方法为手术切除，发生恶变及转移的可能性较低，复发率约10% ～20%，且多发生于术后6 个月以内，定期随访非常重要。

Melanotic neuroectodermal tumor of infancy （MNTI） is an uncommon， rapidly growing neoplasm of neural crest origin that primarily develops in the maxilla of infants during their first year of life， mandibular lesions are rare. We present a case of a newborn with MNTI involving the anterior mandibular region who are treated by a surgical excision of the lesion with safe margins. A review of literature also been done to discuss the clinicopathological features of MNTI. Oral MNTI appear to occur in certain locations and people of certain age. Special attention should be paid to the color of the tumor during intraoral examination. This tumor grew rapidly and invasively. The typical radiographic appearance of MNTI osseous lesions is a well-demarcated radiolucent lesion with bone-expansion. CT image may show bone expansion and destruction， mostly be low density. MRI image usually shows low signal on both the T1 and T2 weighted sequences. Histologically， MNTI was composed of larger melanin-containing epithelial cells and small neuroblast-like cells. Surgical excision is the common treatment for MNTI. In the literature， a few cases of malignancy have been published. However， the rate of recurrences varies between 10% and 20%， in particular within the first 6 postoperative months.Follow-up is highly recommended.