婴幼儿黑色素性神经外胚瘤非常罕见,肿瘤好发于上颌骨,其次为下颌骨、颞部及颅骨等。镜下可见两种类型的瘤细胞,一种为大的胞质内含黑色素的上皮样瘤细胞,排列成腺管样或裂隙状,一种为小的神经母细胞样瘤细胞,呈散在分布或位于腺样结构内。瘤细胞可表达多种神经内分泌细胞、黑色素细胞及上皮细胞的标记。镜下可见上皮样瘤细胞内有黑色素小体,神经母细胞样瘤细胞内有神经内分泌颗粒。本文对该肿瘤的临床病理学特点、病理诊断、鉴别诊断及预后等做一探讨。 Infantile melanotic neuroectodermal is very rare, tumors occur in the maxilla, followed by the mandible, temporal and skull. Microscope shows two types of tumor cells, one is a large cytoplasm containing melanoma epithelioid tumor cells arranged in a duct-like or fissure, a small neuroblastoid tumor cells were scattered Or located within the adenoid structure. Tumor cells can express a variety of neuroendocrine cells, melanocytes and epithelial cells markers. Microscope shows epithelial tumor cells within melanosomes, neuroblastoma cells have neuroendocrine granules. This article on the clinicopathological features of the tumor, pathological diagnosis, differential diagnosis and prognosis to do a discussion.