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. The gene discussed is IDH1; the disease is central nervous system cancer.