Frontal variant of Alzheimer’s Disease is a rare variant of AD (fvAD) associated to a more complex differential diagnosis with respect to behavioral variant of frontotemporal dementia (bvFTD). In these patients, brain atrophy predominantly affects temporoparietal cortex in both classical AD and fvAD compared to bvFTD in which anterior areas are mostly involved. The pattern of hypometabolism is partially overlapping fvAD and FTLD, but semiquantitative tools for the analysis of PET images may be helpful. The use of a pathologic biomarker of amyloidosis (amyloid PET or CSF Aβ-amyloid) is useful to further sustain the diagnosis of fvAD.