The chapter reviews the major clinical syndromes within the frontotemporal dementia (FTD) spectrum and outlines some cross-cultural challenges in the diagnosis of FTD. It describes the epidemiology, pathophysiology, genetics and treatment for FTD. Recent classification guidelines identify three main subtypes of primary progressive aphasia (PPA): non-fluent/agrammatic, semantic and logopenic. Language differences also affect the diagnosis and implementation of specific linguistic tests. Grammatical language differences also pose a challenge, as literal translations of commonly used grammatical tests in the United States can fail to target the intended grammatical demands once translated into Spanish. Cultural perceptions can also affect the ascertainment of familial FTD in Latin America. Management is therefore directed at minimizing symptoms and helping patients and caregivers cope with the impact of the disease. Finally, family education and counseling are crucial in the management of FTD.