Frontotemporal Lobar Degeneration (FTLD) is nowadays considered the second cause of dementia, accounting for 20% of all the cases under the age of 65 years. The earliest signs of frontotemporal dementia are a fundamental change in personality, social behavior, and communication abilities. The pathological and clinical heterogeneity of FTLD has made the application of effective therapies a difficult task. No treatments for FTLD are available yet, and off-label medications are commonly used as symptomatic drugs to counteract behavioral, cognitive and motor disturbances. In general, two different approaches to treat FTLD have been considered. First, treatments to improve clinical symptoms and second, disease-modifying therapies aimed at slowing disease progression by acting on molecular mechanisms, thought to be involved in FTLD pathogenesis. This brief review aims to highlight recent advances in the treatment of FTLD.