Persistent leukocytosis with megalosplenia without any underlying infectious or inflammatory cause is a common manifestation among patients with myeloproliferative neoplasm (MPN), especially chronic myeloid leukemia (CML).[1–3] We report a rare case of myeloid neoplasm with BCR-PDGFRA rearrangement characterized by marked elevation of leukocyte count and megalosplenia, which might improve the focus on this group of diseases and potentially reduce missed diagnoses or misdiagnoses. The gene discussed is PDGFRA; the disease is chronic myelogenous leukemia, BCR-ABL1 positive.