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This review article compiles updated information on Plasmacytoid Dendritic Cell Leukemia, a type of uncommon or rare myeloid neoplasm, with typically cutaneous manifestations with or without extramedullary involvement and leukemic dissemination, which makes it difficult to differentiate from other neoplasms. This review aims to provide a brief update on key points such as its epidemiology, pathogenesis, diagnosis and treatment, in order to allow early recognition of the disease considering its rapid evolution and poor prognosis.