Pathological gambling (PC) is an impulse control disorder characterized by the irresistible urge to gamble, potentially leading to severe personal and social consequences. This disorder has been reported in a few neurological conditions, mainly affecting the dopamine reward pathways. The present chapter reviews the literature on the recent reports of late-onset PC in patients with frontotemporal dementia (FTD). It is suggested that dysregulation of prefrontal cortex and mesolimbic pathways might be implicated in the pathophysiology of gambling behaviors associated with neurodegenerative processes. Specifically, these case reports show that FTD can present with a wide range of behavioural symptoms and should be considered in the differential diagnosis of late-onset PG.