Some subtypes of acute myeloid leukemia share morphologic, immunophenotypic, and clinical features of acute promyelocytic leukemia but lack a promyelocytic leukemia-retinoic acid receptor alpha fusion gene. Herein, we present a case of acute myeloid leukemia with morphological and clinical features resembling those of acute promyelocytic leukemia, carrying the rare DEK::NUP214 fusion gene and presenting with sweet syndrome as the initial manifestation. In our case, the patient with acute myeloid leukemia carrying DEK::NUP214 fusion exhibited highly active bone marrow proliferation, with increased basophil and promyelocyte counts. To the best of our knowledge, this is the first reported case of acute promyelocytic leukemia-like acute myeloid leukemia carrying DEK::NUP214 fusion.