Frontotemporal dementia (FTD) is a progressive neurodegenerative disorder that primarily affects the frontal and temporal lobes of the brain, leading to significant changes in personality, behaviour, and speech. It is the second most common cause of early-onset dementia, typically affecting individuals between the ages of 45 and 65. The aetiology of FTD is highly heterogeneous, with a substantial proportion of cases attributed to genetic mutations (30-50%). The three most commonly implicated genes in familial FTD are MAPT, GRN, and C9orf72. Mutations in the MAPT gene lead to the production of abnormal tau proteins, resulting in the formation of neurofibrillary tangles, which disrupt normal neuronal function. GRN gene mutations cause a reduction in progranulin levels, a protein crucial for neuronal survival and inflammation regulation. Both GRN and C9orf72 gene mutations lead to diverse neuropathological features, including TDP-43 proteinopathies. The hexanucleotide repeat expansion in the C9orf72 gene is the most frequent genetic cause of FTD and is also associated with amyotrophic lateral sclerosis (ALS). Recent advances in genetic research have identified additional genes associated with FTD. Each gene contributes to FTD via distinct pathogenic mechanisms, underscoring the genetic complexity of the disease. Understanding the genetic background of FTD can aid in the development of personalized medicine approaches. Efforts to develop gene therapies are underway, with many novel therapeutic strategies currently in early clinical stages. These developments hold promise for more effective treatments in the future. Genetic counselling and testing are also becoming integral parts of identifying at-risk individuals. In conclusion, the genetic background of frontotemporal dementia involves various genes and pathogenic mechanisms; continued research into FTD is essential for developing targeted therapies and improving outcomes for patients.