目的报道 1例罕见的静脉内子宫平滑肌瘤病病例。　方法通过病史 ,体检、手术所见和病理检查结合文献 ,分析讨论。　结果病理诊断为子宫静脉内平滑肌瘤病伴壁间型平滑肌瘤。　结论本病是一种少见的具有侵袭性生物学行为的良性肿瘤。肿瘤可沿腔静脉生长直至右心房而产生相应症状。可复发并发生肺转移 ,但预后良好。病理上需与内膜间质肉瘤 ,平滑肌肉瘤和子宫肌瘤伴血管浸润行鉴别诊断。 Objective To report a rare case of intravenous uterine leiomyomata. Methods Through the history, physical examination, surgical findings and pathological examination combined with the literature, analysis and discussion. Results Pathological diagnosis of uterine venous leiomyoma with wall-type leiomyoma. Conclusion The disease is a rare benign tumor with invasive biological behavior. Tumor can grow along the vena cava until the right atrium and produce the corresponding symptoms. Recurrence and lung metastasis, but the prognosis is good. Pathological and endometrial stromal sarcoma, leiomyosarcoma and uterine fibroids with vascular invasion line differential diagnosis.