Frontotemporal dementia is a rare form of progressive intellectual deterioration. Its most prominent clinical features are alterations in personality, motivation, and social conduct whereas memory and orientation remain largely unimpaired. Several underlying neurodegenerative processes may be distinguished which are confined to the cerebral cortex in most cases but occasionally involve the basal ganglia and rarely the anterior horn cells. Most frequently, histopathological examination reveals a non-specific loss of neurons accompanied by reactive gliosis. In a minority of cases, globose intraneuronal inclusions and achromatic ballooned neurons are seen. These peculiar morphological changes are called "Pick bodies" and "Pick cells" after the neurologist Arnold Pick who worked in Prague. It can be difficult to identify frontotemporal dementia because its major symptoms mimicnon-organic psychiatric disorders including mania, obsessive-compulsive disorder, schizophrenia, depression or personality disorder. Another problem of diagnosis is that all clinical instruments that are available for assessing cognition, activities of daily living, and non-cognitive symptoms have been tailored to the prototypic dementia in Alzheimer's disease and are less sensitive to the psychopathology of frontal lobe diseases. The burden that frontotemporal dementia imposes on caregivers is also completely different from the one encountered by families of patients with the more prevalent dementias. In the present contribution we summarize the current evidence on epidemiology, aetiology, and risk factors of frontotemporal dementia. Clinical symptoms and course will be illustrated by a case example. We will also provide guidelines for diagnosis and discuss treatment options.