Rosai-Dorfman病(RDD)又称为窦性组织细胞增生伴巨大淋巴结病,是一种病因不明的良性病变,临床极为罕见,可累及全身淋巴系统,也存在结外病变,其中皮肤是最常见的受累部位。颌面部Rosai-Dorfman病可表现为结内型,亦有结外型,其临床特点和影像学表现缺乏特异性,故需病理检查明确诊断,同时术后需要配合免疫治疗及定期随访。本文报告1例颌面部皮肤RDD复发患者,并结合文献对其临床特点、诊疗及预后进行讨论。 Rosai-Dorfman’s disease (RDD), also known as sinusoid cell hyperplasia with giant lymphadenopathy, is a benign disease of unknown etiology and is extremely rare in the clinic and can involve the entire body lymphatic system with extranodal lesions, of which the skin is the most common Affected parts. Maxillofacial Rosai-Dorfman’s disease can be manifested as endoprostate, also have knot appearance, the clinical features and imaging performance of the lack of specificity, it requires a clear pathological examination, at the same time with the need for immunotherapy and regular follow-up. This article reports a case of maxillofacial skin RDD patients, and combined with the literature on its clinical features, diagnosis and treatment and prognosis are discussed.