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Objective: To report the long-term survival of a patient with maternal plasmacytoid dendritic cell tumor (BPDCN) treated by allo-HSCT. Methods: The patient was diagnosed by skin infiltration, bone marrow involvement, skin biopsy and bone marrow cytology. CD4, CD56, and CR123 were expressed in tumor cells. The first complete remission (CR1) was achieved by CHOP-E and MA regimens before transplantation. In March 2018, HLA 5/10 matched hematopoietic stem cell transplantations were performed in the paternal donors and fathers. The pretreatment regimen was FTBI (4 Gy × 2, total lung dose 6 Gy) + CY (cyclophosphamide 1.8 g/m(2) × 2 d) + Flu (30 mg/m(2) × 4 d) + ATG (10 mg/kg); CSA + MMF + MTX to prevent GVHD. MNC 6.45 × 10(8)/kg and CD34 + cells 7.40 × 10(6)/kg were transfused back. + Granulocyte and platelet were engrafted 12 days and 14 days respectively. The donor-recipient chimerism was monitored regularly, immunosuppressive agents were regulated, and minimal residual disease (MRD) was monitored by flow cytometry. No DLI. Results: Complete donor implantation and continuous remission were achieved after transplantation. After transplantation, complications such as mucositis, viral infection, hypoproteinemia, and renal dysfunction occurred. At present, the disease-free survival is 10 months. Conclusion: BPDCN combined with TBI in the CR1 phase can effectively control the disease; HLA haploidentical hematopoietic stem cell transplantation is also an alternative treatment, and complications should be treated in a timely manner.