目的　探讨儿童Erdheim-Chester病（ECD）的临床特点。方法　回顾分析2例ECD患儿的临床表现、诊断 及α干扰素治疗效果，并结合文献进行总结。结果　2例男性患儿，分别为4、 5岁，均表现为多饮、多尿、骨痛、肝脏增大 等，与郎格罕斯细胞组织细胞增生症相似，但病理表现为黄色类脂质肉芽肿，免疫组化中CD1a（－）、S-100（－）。与成人 ECD相比，儿童骨骼系统同样最易受累，神经系统比成人更易受累，心脏、皮肤等受累少于成人。 2例患儿α干扰素治疗 效果尚可。结论　ECD是一种临床上罕见的非郎格罕斯细胞性组织细胞增生症，早期识别、早期诊断可改善预后。

　Objective　To explore clinical characteristics of Erdheim-Chester disease (ECD) in children. Method The clinical manifestations, imaging findings and pathological finding of two childhood ECD patients in our hospital were retrospectively summarized. Results　The most common manifestations of ECD in children included polydipsia, polyuria, lower limb pain, and hepatomegaly, etc., which were similar to Langerhans cell histiocytosis in manifestations, but the pathological features were similar to yellow lipid granuloma, immunohistochemical features are CD68 (+), CD1a (?), and S-100 (?). Compared with that in adults, skeletal systems were also most likely to be involved in children, and nervous system seemed to be more likely to be involved in children, while heart and skin were rarely involved. Two children were treated with interferon with improved clinical situations. Conclusions　ECD is a rare non-Langerhans cell histiocytosis. Early identification and diagnosis can improve the prognosis of the disease.