This paper reports a case of melanotic neuroectodermal tumor of infancy (MNTI)arising in the maxilla of a 3-month-old male infant.The treatment included surgical excision of the lesion with safe margin,curettage of the maxilla and removal of associated developing tooth bud.Microscopically,it proved to be a dual tumor with small,neuroblastic-like cells and larger epithelial cells.Immunohistochemical staining demonstrated epitheloid cells HMB45(+),EMA(+),CK(+);neuroblast-like cells NSE(+),GFAP(+),S-100(+),but both cells Vim(+),CD45(-),Myogenin(-).The 18-mouth follow-up showed no recurrence or metastasis.The related literature was re-viewed.%笔者报告1例婴儿色素性神经外胚层瘤（MNTI）：3月大男婴，发现右上颌骨处肿物半月，行肿物切除术，并刮除累及牙胚和骨质。镜下示肿瘤含上皮样细胞和神经母细胞，免疫组化示上皮样细胞 HMB-45、CK、EMA、Vim 阳性，神经母细胞 S100、NSE、GFAP、Vim 阳性。术后18个月密切随访，未发现复发转移。对相关文献进行了复习讨论。
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