OBJECTIVES: Patients with dementia show dissociations between musical semantic memory (i.e., spared musical lexicon) and other memory modalities, except in some severe cases. We aim to study, from a neuropsychological point of view, the dissociation between musical semantic memory compared to language and verbal memory in a patient with severe Behavioral Variant Frontotemporal Dementia (bvFTD). We hypothesize a single dissociation between these domains will be found, with sparing of musical semantic memory. METHODS: LC, a patient with severe bvFTD, and three matched controls were assessed through language, semantic, and episodic memory, and musical semantic memory tasks. The control group had similar music taste as LC: to participate as controls, RESULTS: LC showed impairment in all Verbal Memory tasks, but not in musical tasks. There was a dissociation between musical semantic memory, and language and verbal semantic memory. CONCLUSIONS: The musical lexicon can be preserved in advanced stages of dementia, which supports the idea that music can be a therapeutic tool in patients with severe dementia.