目的：探讨小儿先天性中胚叶肾瘤（ＣＭＮ）的临床特点、诊断及治疗。方法：近２０年间收治ＣＭＮ７例，男４例，女３例，年龄７个月～１０岁。术前均可扪及一腹部肿块，其中１例伴肉眼血尿，病变位于左侧４例，右侧３例。全部病例均接受了手术治疗，２例证实有肾门旁肿瘤转移，病理检查属平滑肌瘤型５例，细胞型２例，因术前及术中疑诊为Ｗｉｌｍ＇ｓ瘤而接受了不同疗程化疗。结果：除１例２个月龄患儿术后死于窒息外，余６例术后均随访，１例术中肿瘤破裂者术后８个月复发，化疗无效后再次手术已无瘤生存９年，余５例无瘤生存至今，最短５年，最长已１５年。结论：ＣＭＮ是一罕见的肾肿瘤，虽好发于新生儿及婴儿早期，但也可见于年长儿，其临床表现和病理改变虽有诸多特点，但有时仍难与Ｗｉｌｍ＇ｓ瘤区别，手术切除是治疗本病最有效的方法 Objective: To investigate the clinical features, diagnosis and treatment of congenital mesodermal nephroma (CMN) in children. Methods: CMN7 cases were treated in the past 20 years, 4 males and 3 females, aged 7 months to 10 years old. Preoperative palpable abdominal mass, including 1 case of gross hematuria, lesions in the left 4 cases, 3 cases of right. All cases were treated surgically, 2 cases confirmed metastasis of renal cell carcinoma, the pathological examination was 5 cases of leiomyoma, 2 cases of cell type, due to preoperative and intraoperative suspicion of Wilm’s tumor and accepted a different Course of chemotherapy. Results: Except 1 case of 2-month-old children died of asphyxiation after operation, the remaining 6 cases were followed up. One case of tumor rupture recurred 8 months after the operation. The tumor was re-operative after the ineffective chemotherapy. 9 Year, more than 5 cases of tumor-free survival so far, the shortest 5 years, the longest has 15 years. CONCLUSIONS: CMN is a rare renal tumor that occurs in early neonates and infants but is also seen in older children. Although its clinical manifestations and pathological features are numerous, it is sometimes difficult to distinguish it from Wilm’s tumor, Surgical resection is the most effective way to treat this disease