Objectives: Frontotemporal dementia (FTD) is the second most common form of degenerative dementia in early onset dementia (EOD) patients. The core disorders in the FTD spectrum are: behavioral variant of FTD (FTDbv) with predominant early changes in behavior (such are disinhibition, apathy, loss of empathy, compulsive behaviour..); and language variants - primary progressive aphasias (PPA) where language is the primary impairment (difficulties in speech, understanding, repetition..). Patients with sporadic, as well as genetic forms of FTD, can develop motor symptoms of motor neuron disease (before, after or at the same time with cognitive/behavioural features) or atypical parkinsonism-progressive supranuclear palsy or corticobasal syndrome, which are all part of the clinical FTD spectrum. The FTD is still poorly recognised entity and the heterogeneity of clinical presentations, early onset (most frequently before the age of 65), as well as overlap of early FTDbv symptoms and psychiatric diseases, often results in wrong diagnosis and presents a challenge, even in tertiary referral centres. It has been shown that delay in correct dementia diagnosis contributes to the caregiver's and patient's distress. The early and precise diagnosis is important for consideration of prognosis and course of the disease with family members; possibilities of improving patient's quality of life by giving them adequate symptomatic therapy; guiding the genetic analysis which is especially important for family members who also could be in risk of carring or passing the mutation to their offspring. Aim: In this mini review paper we tried to point out the specificity of clinical manifestation in the FTD spectrum, which could be helpful in making the early and accurate diagnosis among the variety of EOD cases. Conclusion: Early and accurate FTD diagnosis is important for the insight of the disease course, giving adequate symptomatic therapy and guiding the genetic analysis which reduces the caregiver's and patient's distress
