外毛根鞘癌首先由Headington1976年提出,是一种罕见的皮肤附属器外毛根鞘肿瘤。一般认为增生性外毛根鞘瘤为化学刺激、感染、外伤等导致外毛根鞘鞘内容物的增生,多数报告病例出现在老年人长毛发的解剖部位。尽管多数显示良性肿瘤的临床进展,但是有报道显示肿瘤局部活性升高,甚至局部扩大切除后具有潜在转移的风险。我科收治1例发生于颌面部巨大恶性增殖性外毛根鞘瘤病例,报告如下: External hair root sheath cancer first introduced by Headington in 1976, is a rare skin appendage sheath root sheath tumors. Generally believed that the proliferation of hairy root sheath tumors for chemical irritation, infection, trauma and other outer sheath root sheath lead to the proliferation of contents, the majority of reported cases appear in the elderly hair of the anatomical site. Although most of them show the clinical progress of benign tumors, it has been reported that local activity of the tumor is increased, and there is a risk of potential metastasis even after local expansion and excision. One case of our department admitted to the maxillofacial malignant proliferative outer root hamstrings cases, the report is as follows: