Abstract: Objective To improve the understanding of blastic plasmacytoid dendritic cell neoplasm (BPDCN) by reporting a case of BPDCN and reviewing its literature. Methods Clinical data about 1 case of BPDCN, including its clinical features and laboratory fi ndings, were analyzed with its relative literature reviewed. Results Pathology and immunology testing showed that the fi rst symptom of BPDCN was skin lesion which was manifested as characteristic nodules and erythema. The diagnosis of BPDCN could be established according to the morphology of marrow cells. Conclusion BPDCN is highly aggressive and progresses rapidly with no effective treatment available at present and a poor prognosis. It is dif fi cult to differentiate it from other acute leukemia only according to the morphology of marrow cells. Its diagnosis can be established according to its clinical manifestations combined with pathology and immunology testing.