In the present study we aimed at evaluating the prevalence and the associated clinical, neuropsychological and behavioral features of extrapyramidal symptoms in Frontotemporal Dementia (FTD) patients. Seventy-five patients fulfilling clinical criteria of FTD were consecutively enrolled. Each patient underwent clinical and extrapyramidal sign examination, and neuropsychological and behavioral disturbance evaluation. Each patient was submitted to both brain MRI and SPECT, documenting frontotemporal atrophy/hypoperfusion pattern. Parkinsonian symptoms in FTD were associated with a specific endophenotype characterized by higher incidence of psychotic symptoms, memory deficits and psychomotor speed ability abnormalities. Careful description of the spectrum of presentation of FTD may be of help for further understanding the underpinnings of the disease.