目的：探讨血管肌纤维母细胞瘤(angiomyofibroblastoma, AMF)的临床病理特征、鉴别诊断以及诊治方法和预后。方法：选择青岛大学附属医院医院收治的1例女性外阴血管肌纤维母细胞瘤的病例，进行临床特点、影像学特点、组织形态学、免疫组织化学研究。结合文献分析本病的临床表现、病理形态特点及鉴别诊断。结果：外阴血管肌纤维母细胞瘤具有上皮样细胞，梭形细胞和浆细胞，细胞核多为圆形或卵圆形，核分裂象罕见。病理切片特点为密集细胞和稀疏细胞交替分布，血管分布和肌成纤维细胞的分化明显。免疫组织化学：肿瘤细胞Desmin，CD34，Vimentin，ER均阳性。结论：外阴血管肌纤维母细胞瘤是一种极少见的特殊的外阴部良性软组织肿瘤，临床诊断较为困难。治疗以手术为主，术后仍需复查。 Objective: To investigate the clinicopathological features, differential diagnosis, treatment and prognosis of angiomyofibroblastoma (AMF) in vitro. Methods: The clinical features, imaging features, histomorphology and immunohistochemistry of a case of female vulvar vascular myofibroblastoma were studied. The clinical manifestations, pathological features and differential diagnosis of the disease were analyzed. Results: AMF has epithelioid cells, spindle cells and plasma cells. The nucleus is mostly round or oval, and nuclear mitosis is rare. The pathological sections were characterized by alternating distribution of dense cells and sparse cells, vascular distribution and differentiation of myofibroblasts. Immunohistochemistry showed that positive reaction of tumor cell were seen with Desmin, CD34, Vimentin, ER. Conclusions: Vulvar vascular myofibroblastoma is a rare and benign tumor of soft tissue in vitro. The surgical treatment is the main method, and postoperative re-examination is still required.