Abstract: Peutz-Jeghers syndrome（PJS）is an autosomal dominant disorder caused by pathogenic variants in the STK11 gene，characterized by mucocutaneous pigmentation，gastrointestinal hamartomatous polyps，and an increased susceptibility to cancer. In pediatric patients，complications due to gastrointestinal polyps are the primary factors that threaten the life and health of PJS children. Currently，many challenges remain in the clinical management of PJS，including delayed diagnosis，high rates of complications，and non-standardized management approaches.This review focuses on early detection of PJS in children，management strategy of polyps，tumor monitoring protocols，and key points in familial management，aiming to improve the long-term prognosis of PJS children by emphasizing the importance of standardized，whole-process management.

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摘要： Peutz-Jeghers综合征（Peutz-Jeghers syndrome，PJS）是一种由STK11基因突变引起的常染色体显性遗传病，临床特征主要包括皮肤黏膜色素沉着、胃肠道错构瘤性息肉以及显著的肿瘤易感性。在儿童期，胃肠道息肉引发的并发症是威胁PJS患儿生命健康的首要因素。当前，PJS的临床管理仍面临诸多挑战，如诊断延迟、严重并发症发生率居高不下、治疗方案不规范等问题。该文系统阐述了儿童PJS的早期识别、息肉管理策略、肿瘤监测方案及家系管理要点，旨在强调全流程规范化管理的重要性，从而改善PJS患儿的长期预后。

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