The paper reviews convergent evidence on the ability to attribute mental states to one's self and to others (i.e., theory of mind, ToM) in patients affected by the behavioural variant of frontotemporal dementia (bv-FTD). This disease represents a particular challenge for researchers and clinicians, due to its insidious onset and ambiguous clinical features, which frequently render difficult a precise and timely diagnosis. The paper proposes a way to shed new light on the hypothesis that the neuropsychiatric profile of individuals with bv-FTD can be at least partially explained by a deficit in ToM ability. We examined both neuroimaging data on the neural correlates of ToM ability in healthy participants and studies investigating the progressive cerebral atrophy in patients with bv-FTD. Our findings suggest a link between the progressive degeneration of the anterior regions of medial frontal structures characterising the early stages of the bv-FTD and the ToM deficit these patients show. They also suggest the importance of using ToM tests during the diagnostic process of bv-FTD.