Herein, we report a case of myeloid neoplasm with a rare variant translocation, t(4;22)(q12;q11), involving the <i>BCR::PDGFRA</i> fusion gene and coexisting <i>PDGFRA</i> variants, accompanied by persistent leukocytosis, massive splenomegaly, and eosinophilia. This evidence concerns the gene PDGFRA and Splenomegaly.