Abstract. Cutaneous and systemic plasmacytosis is an exceedingly rare condition that is identified in Japanese individuals in particular. The present study describes the case of a patient of mainland Chinese origin who manifested with red-brown macules, papules and plaques limited to the face. Identifying a therapy for cutaneous and systemic plasmacytosis is quite difficult, however, the present patient showed a good response to low-dose thalidomide. The exact mechanism of action is not yet clear, however, we hypothesize that thalidomide may function through decreasing the secretion of interleukin-6 and affecting the growth of plasma cells.