<h4>Background</h4>Myeloproliferative neoplasms (MPNs), typically defined by myeloid proliferation and eosinophilia, and are only rarely caused by platelet-derived growth factor receptor beta (PDGFRB) gene rearrangements.<h4>Case presentation</h4>Here, we report a unique case of MPN that is negative for eosinophilia and characterized by a novel <i>PDGFRB</i> rearrangement. Here, PDGFRB is linked to myeloproliferative neoplasm.