目的：通过核查某院近10年黑斑息肉综合征ICD⁃10编码，分析编码错误原因，探讨改进思路，提高罕见病编码正确率。方法：利用病案统计系统以及病理质控与管理系统，调取某院2012年1月1日⁃2021年12月31日黑斑息肉综合征病例进行回顾性分析，经病理科质控员和临床医师确认为黑斑息肉综合征93例，由编码员对编码进行审核。结果：93份病历中正确编码68例，占比73.1%；错误编码25例，占比26.9%。其中错编成息肉14例，占比56%；编成良性肿瘤3例，占比12%；编成后天性小肠畸形2例，占比8%；漏编码2例，占比8%；其它4例（编成错构瘤病、家族性息肉病、消化道多发息肉综合征、胃结石各1例），占比16%。结论：黑斑息肉综合征编码具有一定的难度，准确编码需要编码员和临床医师们共同完成。临床医师和编码员应该有此疾病相应的知识储备，积极与病理科及时沟通。编码员应熟练掌握编码技能和具备独立思考的职业习惯，提高编码正确率。

Objectives：Through checking the ICD⁃10 coding of Peutz⁃Jeghers Syndromein the past 10 years，the causes of coding errors were analyzed，and the improvement ideas were discussed to improve the coding accuracy of rare diseases. Methods：Using the statistical system of medical records and the pathology quality control and management system，the cases of Peutz⁃Jeghers Syndrome from January 1，2012 to December 31，2021 in acertain hospital were collected for retrospective analysis.93 cases of Peutz⁃Jeghers Syndrome were confirmed by the quality control officer of pathology department and clinicians，and than the codes were reviewed by the coders. Results：Among 93 medical records，68 cases were correctly coded，accounting for 73.1%. There were 25 cases of incorrect coding，accounting for 26.9%. Among them，14 cases were miscoded into polyps，accounting for 56%. 3 cases were classified as benign tumors，accounting for 12%. 2 cases were acquired small bowel malformation，accounting for 8%. There were 2 cases of missing coding，accounting for 8%. The other 4 cases were miscoded into hamartomatosis，familial polyposis，multiple polyps syndrome of digestive tract and gastric calculi，accounting for 16%. Conclusions：The coding of Peutz⁃Jeghers Syndrome is difficult. Accurate coding requires both the coder and the clinician. Clinicians and coders should have the corresponding knowledge reserve of this disease and actively communicate with the pathology department in time. Coders should master coding skills and have the professional habit of independent thinking to improve coding accuracy.