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Abstract Background Lewy Body Dementia (LBD) is a complex neurodegenerative disorder marked by fluctuating cognition, parkinsonism, and visual hallucinations. Despite being the second most common form of dementia, it remains misunderstood and underdiagnosed. Existing diagnostic and therapeutic approaches rarely account for sex and gender differences, which may contribute to delayed diagnosis and suboptimal care, particularly for women. Understanding how sex and gender interact with LBD mechanisms and patient experiences is essential for developing equitable, person-centred interventions. Methods This study employed a structured evidence synthesis, drawing from peer-reviewed literature on LBD with a focus on sex- and gender-related variables. A comprehensive search across PubMed, Scopus, and Web of Science identified original research, systematic reviews, and meta-analyses examining biological (e.g., hormonal, genetic, and neurochemical) and gender-related (e.g., caregiving roles, health-seeking behaviours, stigma) factors in LBD. Data were analysed through narrative synthesis, triangulating findings across clinical presentation, lived experiences, and pathophysiological mechanisms. Results Males with LBD are more likely to exhibit parkinsonian symptoms and visual hallucinations, whereas females face greater mood disturbances and caregiver-related stress; moreover, diagnostic tools tend to underrepresent female symptomatology. Our findings reveal that, contrary to previous assumptions of uniformity, there are substantial sex and gender-based variations in LBD’s pathophysiology and patient experience, impacting both disease recognition and management. This adds to prior knowledge by demonstrating that both biological and sociocultural factors must be considered to improve outcomes. Conclusion Sex and gender are fundamental, yet underexplored, dimensions of LBD. This paper proposes a conceptual framework integrating biological sex factors with sociocultural gender influences. By examining their interplay, our perspective highlights the importance of integrating sex and gender into LBD research and care. This approach not only improves diagnostic accuracy and treatment effectiveness, but also advances equity and strengthens support systems for both patients and caregivers.