Abstract: This paper explores the role of general practitioners（GPs） in the management of rare diseases through the analysis of the problems faced by patients in the course of the diagnosis and treatment of a rare disease case.According to the core competence and characteristics of GPs proposed by WONCA，this paper puts forward that GPs can play an important role not only in the field of the diagnosis and treatment of common and frequently-occurring diseases，but also in the management of all diseases throughout the whole life cycle of human beings.

Key words: Rare disease, Histiocytic sarcoma, General practitioners, WONCA, Disease management, Ability, Effect

摘要： 本文通过1例罕见病案例诊疗过程中患者面临的问题，分析全科医生在罕见病管理中的作用，根据世界家庭医生组织（WONCA）提出全科医生的核心能力和特质，提出全科医生不仅在常见病、多发病的诊疗领域，而且能在人的整个生命周期全病种的管理中发挥重要作用。

关键词: 罕见病, 组织细胞肉瘤, 全科医生, 世界家庭医生组织, 疾病管理, 能力, 作用