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目的:探讨不同分型儿童颅咽管瘤的临床特点、手术方式及疗效。方法:回顾性分析华中科技大学同济医学院附属同济医院神经外科2006年3月至2018年7月经手术治疗的103例儿童颅咽管瘤的临床资料。根据鞍膈和脑室为界，将儿童颅咽管瘤分为鞍内型、鞍上型和脑室内型三种类型(即三组)。结果:103例患儿中，鞍内型34例，鞍上型63例和脑室内型6例。三组在患儿年龄、性别、肿瘤体积、影像学和病理学特点、肿瘤全切率和围手术期病死率等资料的差异均无统计学意义(n P>0.05)。本组病例总体手术全切率为88.3%(91/103)，围手术期病死率为5.8%(6/103)。各组患儿的手术入路、肿瘤复发率和预后的差异均具有统计学意义(n P<0.05)。鞍内型患儿选择经蝶入路或翼点入路，鞍上型患儿选择翼点或经纵裂入路，脑室内型患儿选择皮层/纵裂-侧脑室入路。对所有患儿随访(32.3±25.9)个月，总体肿瘤复发率为16.5%(17/103)，其中鞍内型经蝶手术后复发率显著高于其他两组(n P<0.05)；总体预后良好率为88.7%(86/97)，鞍内型和鞍上型颅咽管瘤患儿预后较好，脑室内型预后差(n P0.05). The total resection rate was 88.3% and perioperative mortality 5.8%. Microsurgical approach, recurrence rate and prognosis were analyzed statistically for various types of craniopharyngiomas. The transsphenoidal and pterion approaches were selected for infradiaphragmatic craniopharyngiomas, pterion and longitudinal approach for supradiaphragmatic craniopharyngiomas and cortex/longitudinal-ventricle approach for intraventricular craniopharyngiomas. patients were followed up for (32.3±25.9) months in all cases. Seventeen patients were diagnosed as tumor recurrence with a recurrence rate of 16.5%(17/103). Transsphenoidal microsurgery of infradiaphragmatic craniopharyngiomas resulted in a higher tumor recurrence as compared with other types (n P<0.05). The favorable prognostic rate was 88.7%(86/97) and infradiaphragmatic and supradiaphragmatic craniopharyngiomas fared better than intraventrical tumors.n Conclusions:Clinical classifications of craniopharyngiomas may indicate tumor location and growth pattern directly and offer guidance for microsurgical approach selection, tumor recurrence and prognostic prediction.