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Objective To investigate the feature,pathological diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN).Methods The clinical and histological findings,immunophenotype,treatment and prognosis of one case with BPDCN were evaluated with review of the relevant literature.Results The female patient with age of 32 years presented as cutaneous lesions.Pathologic findings showed skin and bone marrow were involved.Microscopically,the tumors were composed of diffuse blasts with small to medium size,scant cytoplasm,highly irregular nuclear contours,dispersed nuclear chromatin and faint indistinct nucleolus.Immunohistochemical characteristics of the tumors were the positive expression of CD4,CD56 and CD123,and negative expression of CD3,CD20,CD34,TdT,MPO.Conclusion BPDCN is a rare and highly aggressive hematopoietic neoplasm.Except for the skin,bone marrow and lymph node can also be involved.The differential diagnosis is necessary from other haematopoietic and lymphoid tumors.