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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and highly aggressive hematopoietic malignancy associated with a poor prognosis. It has been recognized to originate from precursors of plasmacytoid dendritic cells and has recently been established as a distinct entity. The most frequent clinical presentations are prominent skin lesions, followed by peripheral blood, bone marrow, and other organ involvement. Treatment outcomes are often disappointing due to a high relapse rate after chemotherapy and risk of leukemic dissemination. Herein, we report a case of BPDCN in a 41-year-old man who presented with cutaneous and nasopharyngeal lesions, and achieved a complete response after systemic chemotherapy. Dermatologists should be aware of BPDCN and its clinical presentations, as early diagnosis with appropriate treatment is crucial to improve its prognosis.