Objective: Individuals with high levels of cognitive reserve manifest less severe clinical symptoms early in the neurodegenerative disease course. In the behavioral variant of Frontotemporal Dementia (bvFTD), neuropsychiatric symptoms typically emerge before significant cognitive deficits can be detected. This has implications for timely and accurate diagnosis among individuals with high cognitive reserve. This case study highlights challenges of early diagnosis in bvFTD, such as diagnostic accuracy and limitations of neuropsychological test sensitivity in relation to high cognitive reserve in a male physician, offering suggestions for improving early detection. Method: A 66-year-old, Hispanic male physician underwent brain MRI, PET imaging, and psychiatric, neurological, and neuropsychological testing. Symptom onset 8 years ago was characterized by: personality changes, subtle disinhibition, frivolous spending, anger outbursts, and risk-taking behaviors. Recently, mild expressive language difficulties and forgetfulness were endorsed, with minimal functional deficits. Results: Psychiatric evaluation confirmed an exacerbation of mood and personality changes with potentially high economic and legal consequences. Neurocognitive performance demonstrated mild decline on measures of attention, language, and delayed memory. Immediate verbal memory and recognition was commensurate with premorbid abilities. PET imaging revealed decreased metabolic activity involving frontotemporal lobes and MRI indicated moderate central and cortical atrophy and chronic ischemic small vessel disease. Disease course, clinical evaluation, neuropsychological testing and brain neuroimaging confirmed FTD. Conclusion(s): Case description highlights the utility of a comprehensive approach in accurately diagnosing individuals with high cognitive reserve, where early disease detection becomes more challenging, and emphasizes the advantages/limitations of neuropsychological assessment in detecting subtle cognitive changes in bvFTD.