In only three cases, molecular analysis was performed, and all of them (100%) exhibited the IDH1 R132H mutation simultaneously in enchondromas and in glioma [8,37,38]; in the unique case of the OD patient affected by acute myeloid leukemia [6], molecular studies were not performed; finally, in our case, the unique patient hosting OD, AML, and BG reported the IDH1 R132H mutation in enchondromas, AML, and brain glioma. This evidence concerns the gene IDH1 and acute myeloid leukemia.