Due to the early and prominent behavioral changes which characterize behavioral variant frontotemporal dementia (bvFTD), patients are more likely to seek psychiatric help and are often initially diagnosed with a primary psychiatric disorder (PPD). Differentiating these conditions is critical because of the dramatically different outcomes, differences in patient management, family counseling and caregiver education. To propose a practical guide to distinguish between bvFTD and PDD. We conducted a non-systematic review of the published manuscripts in the field, including some previous investigations from our own group and work on which we have collaborated, and summarized the main findings and proposals that may be useful for neurological practice. The reviewed literature suggests that a comprehensive clinical history, brief cognitive and neuropsychological evaluations, detailed neurological examination with special attention to motor alterations related to bvFTD, structural and functional neuroimaging evaluation, genetic investigation in selected cases, and assistance from a multidisciplinary team, including a neurologist and a psychiatrist with expertise in bvFTD, are very helpful in differentiating these conditions. Although the clinician may commonly face great difficulty in differentiating between bvFTD and PPD, the use of appropriate tools in a systematic way and the availability of a well-trained multidisciplinary group can significantly increase diagnostic accuracy.