A 71-year-old male diagnosed with myeloid sarcoma had an abnormal cytogenetic profile, with loss of chromosome 7 and evidence of tetraploidy observed for MLL, AML, ETO, BCR and ABL. The patient’s sample was screened on a 54 gene myeloid NGS panel, and was reported to have IDH2 p.R140Q, JAK2 p.V617F, and SRSF2 p.P95L variants. Here, IDH2 is linked to acute myeloid leukemia.