Abstract Background Parkinson’s Disease (PD) is a motor neurodegenerative disorder of the dopaminergic neurons in the substantia nigra 1 . One of the frequent complications is dementia and often is a late symptom 1,2 . Some studies suggest that around 10% of patients with PD develop dementia per year 2,3 . Mutations in PLA2G6 are responsible of PARK14, an autosomal recessive L‐DOPA responsive dystonia/parkinsonism with early/adult onset 4 . This phenotype presents a high clinical variability, which consists in the occurrence of cerebral and cerebellar atrophy, iron accumulation in the basal ganglia, and cognitive decline 4 . In this study, we describe the genotype of two brothers diagnosed with early onset PD and a phenotype with FTD‐like characteristics. Method A complete clinical evaluation consisting of geriatric, psychyatric, neurologic and neurpsychologic evaluation, was carried out in two affected male individuals, and their family. NGS was performed (PD targeted panel, WES, WGS) and MLPA on the two affected individuals. Result The two affected individuals were diagnosed around their thirties, presenting classic motor symptoms of PD. Subsequently, dementia associated to frontotemporal disease was diagnosed. No iron accumulation in the basal ganglia was found in either of the affected siblings. Genetic tests showed the pathogenic variant in heterozygous state c.2132C>G, p.Pro711Arg in PLA2G6 gene in the two affected brothers and their healthy mother. Conclusion Atypical frontotemporal dementia in two siblings with early‐onset PD is described. Additional studies should be performed to identify another variant or another gene in this family. Some studies have risen the hypothesis that even heterozygous PLA2G6 mutations may cause PARK14 4 . References: 1. Hanagasi HA, Tufekcioglu Z, Emre M. Dementia in Parkinson’s disease. J Neurol Sci . Mar 15 2017;374:26‐31. https://doi.org/10.1016/j.jns.2017.01.012 2. Garcia‐Ptacek S, Kramberger MG. Parkinson Disease and Dementia. J Geriatr Psychiatry Neurol . Sep 2016;29(5):261‐70. https://doi.org/10.1177/0891988716654985 3. Aarsland D, Kurz MW. The epidemiology of dementia associated with Parkinson’s disease. Brain Pathol . May 2010;20(3):633‐9. https://doi.org/10.1111/j.1750-3639.2009.00369.x 4. Ferese R, Scala S, Biagioni F, et al. Heterozygous PLA2G6 Mutation Leads to Iron Accumulation Within Basal Ganglia and Parkinson’s Disease. Front Neurol . 2018;9:536. https://doi.org/10.3389/fneur.2018.00536