Blastic plasmocytoid dendritic cell neoplasm is one of the aggressive hematopoietic malignancy with predilection for the skin. It is a tumor derived from the plasmocytoid dendritic precursor cells. Skin is its most common location; however, it may also affect bone marrow, lymph nodes, or spleen. The condition is rarely diagnosed in children; so far only 36 cases have been reported. The diagnosis usually takes time and there are no uniform treatment guidelines. This paper presents a case of a 6-year-old boy with blastic plasmocytoid dendritic cell neoplasm (skin and myeloid location). He has been treated according to the acute lymphoblastic leukemia treatment protocol.
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