Abstract Background The frontal variant of Alzheimer’s disease is characterized by behavioral changes and executive deficits in the early stages. This phenotype is similar in the behavioral variant of frontotemporal dementia, hindering the clinical diagnosis. The aim of this study is to describe the profile of cognitive, behavioral and motor disorders in a family with an autosomal dominant inheritance pattern of early‐onset dementia. Method Five cases of affected individuals of a Colombian family are presented. We conducted an interdisciplinary clinical evaluation involving different specialties including geriatric, neurology, psychiatry, neuropsychology, and genetics. After each individual evaluation, a consensus was reached among the specialists to define the diagnosis. Result In all cases, reported age of onset was before 60 years old. Three out of four cases met criteria for severe dementia and the remaining case presented Mild Cognitive Impairment (MCI). In two cases, the first symptoms reported were memory and planification problems that affected daily life activities. Nevertheless, at the formal evaluation, predominant changes at the executive functions and social cognition were found. In the other two cases, given the severity of the alterations, the evaluation was limited to the analysis of the clinical history, and it is suspected to be cases of linguistic variants of FTD. Conclusion Intrafamilial phenotypic variability was described in this family. This variability in the presentation of the disease phenotype could guide the clinical diagnosis. References: Ossenkoppele, R., Singleton, E. H., Groot, C., Dijkstra, A. A., Eikelboom, W. S., Seeley, W. W., … & Pijnenburg, Y. A. (2022). Research criteria for the behavioral variant of Alzheimer disease: a systematic review and meta‐analysis. JAMA neurology, 79(1), 48‐60. Musa, G., Slachevsky, A., Muñoz‐Neira, C., Méndez‐Orellana, C., Villagra, R., González‐Billault, C., … & Lillo, P. (2020). Alzheimer’s disease or behavioral variant frontotemporal dementia? Review of key points toward an accurate clinical and neuropsychological diagnosis. Journal of Alzheimer’s disease, 73(3), 833‐848.