Multiple gliomas are rare glial tumors with a histology that is typically consistent with high-grade gliomas. A distinction is made between multifocal and multicentric gliomas according to criteria of anatomical continuity, as well as between synchronous and metachronous gliomas according to chronological time of onset. We present the case of a professional saxophonist with a left temporoinsular lesion who underwent awake craniotomy with monitoring of verbal and musical ability as well as primary sensory and motor cortices. Histopathological study revealed an isocitrate dehydrogenase 1 (IDH)-mutant diffuse astrocytoma. After 4 years of complete oncological remission, the patient developed impaired proprioception in all four extremities. An intramedullary lesion was detected at the level of C4 consistent with an IDH wild-type diffuse astrocytoma. We highlight the singularity of this case as it involved two low-grade glial lesions, separated in time (metachronous) and location (multicentric), as well as genetic differences between both lesions (IDH mutant and wild type).