颅内生殖细胞肿瘤是儿童较为少见的颅内肿瘤，其好发于松果体区、蝶鞍区、基底神经节区。根据其组织类型可分为生殖细胞瘤、畸胎瘤、胚胎癌、内胚窦瘤、绒毛膜上皮癌及混合性生殖细胞肿瘤等。肿瘤的临床表现主要与其位置、大小相关，可能出现的内分泌相关症状包括尿崩症、性早熟、生长受限等；治疗主要是放、化疗结合；治疗后可能出现的并发症包括垂体功能减退症、尿崩症、矮小、性发育迟滞等，这些并发症主要采用相关激素替代治疗。该文就儿童生殖细胞肿瘤内分泌问题的现状及研究进展予以综述。“,”Intracranial germ cell tumors are rare in children with a tendency to appear in pine-al,suprasellar and basal ganglia region.They can be classified as germinoma,teratoma, embryona carcinoma,endodermal sinus tumors, choriocarcinoma, mixed germ cell tumor according to their pathohistology.The main clinical manifestations of the tumors depend on their size and location .En-docrine manifestations such as diabetes insipidus ,precocious puberty,and growth retardation can ap-pear during the courses.The tumor therapy regimen is mostly chemoradiotherapy , however endocrine complications including hypohypophysism, diabetes insipidus, short stature and sexual retardation can sometimes appear.Treatments of these complications are always replacement therapy .