Plasmacytoid blast dendritic cell neoplasia (NCDBP) is an uncommon malignant neoplasm, presenting clinically with cutaneous involvement and subsequent lymph node and bone marrow extension. It characteristically expresses the markers: CD56, CD4, and CD123. There is no optimal treatment, relapses are frequent, and the survival time is short. We present the case of an elderly patient with NCDBP who initially presented with cutaneous lesions, but experienced rapid systemic progression and did not, respond to treatment.