先天性静脉畸形骨肥大综合征为一种复杂且罕见的先天性肢体发育畸形性疾病,该疾病以其血管畸形、骨肥大和软组织增生三联征为主要表现。本文通过其发病机理和临床表现,分析病例内容,探讨该疾病的ICD-10编码。按照国际疾病的分类和查找方法,甄选和变通主导词进行查询。分别以综合征、异常、畸形为主导词进行编码的查找,未获得准确编码;以音译词克利佩尔-特脑纳-韦伯综合征为主导词,得到其编码Q87.2,核对卷一,确认编码。通过对疾病编码的讨论分析,总结了罕见疾病的编码思路。编码人员除了坚持学习和巩固编码原则外,还应深入研究疾病诊断的内涵,通过查阅文献寻找切实依据,并与临床医师互相交流,才能编码科学、准确。 Congenital venous malformations Bone hypertrophy is a complex and rare congenital malformation of limb disease that is characterized by vascular malformations, osteoarthritis and soft tissue hyperplasia. This article through its pathogenesis and clinical manifestations, analysis of case content, to explore the disease ICD-10 coding. In accordance with the classification of international diseases and search methods, selection and adaptation of the leading query. Respectively, with syndromes, abnormalities, deformities as the leading code to find the search, not accurate coding; with the transliteration of kelipper - 特纳纳 - Weber syndrome as the leading word, its code Q87.2, check the volume one, Confirm the code. Through the discussion and analysis of disease codes, the coding ideas of rare diseases are summarized. In addition to insisting on learning and consolidating coding principles, the coders should also thoroughly study the connotation of disease diagnosis, seek scientific basis through consulting literature, and communicate with clinicians in order to code scientific and accurate.