To expand the current knowledge of the ETV6-ACSL6 fusion gene, we reported a patient with the ETV6-ACSL6 fusion gene who presented with chronic eosinophilic leukemia, not otherwise specified (NOS), who importantly received proper treatment regimens and consequently had a good prognosis. This evidence concerns the gene ETV6 and Chronic Eosinophilic Leukemia, Not Otherwise Specified.