Abstract: Hypohidrotic ectodermal dysplasia is one of the genetic diseases with typical oral clinical symptoms. Patients come to the department of prosthetics or orthodontics because of tooth agenesis. Therefore，understanding hypoplastic ectodermal dysplasia has certain clinical significance for dentists. This article introduces the etiology，classification，clinical symptoms and treatment of dystrophic ectodermal dysplasia.

Key words: hypohidrotic ectodermal dysplasia, tooth agenesis, genetic disease

摘要： 少汗型外胚层发育不良是一种具有典型口腔临床症状的遗传性疾病，患者常因存在缺牙症状于口腔修复科或正畸科就诊。因此，了解少汗型外胚层发育不良对于口腔医生具有一定的临床意义。文章就少汗型外胚层发育不良的病因、分类、临床表现及治疗方法做一概述。

关键词: 少汗型外胚层发育不良, 牙齿先天缺失, 遗传病