In this review, we summarize the current knowledge on sex differences in dementia with Lewy bodies (DLB) relating to epidemiology, clinical features, neuropathology, biomarkers, disease progression, and caregiving. While many studies show a higher DLB prevalence in men, this finding is inconsistent and varies by study approach. Visual hallucinations may be more common and occur earlier in women with DLB, whereas REM sleep behavior disorder may be more common and occur earlier in men. Several studies report a higher frequency of parkinsonism in men with DLB, while the frequency of fluctuations appears similar between sexes. Women tend to be older, have greater cognitive impairment at their initial visit, and are delayed in meeting DLB criteria compared to men. Women are also more likely to have Lewy body disease with co-existing AD-related pathology than so-called "pure" Lewy body disease, while men may present with either. Research is mixed regarding the impact of sex on DLB progression. Biomarker and treatment research assessing for sex differences is lacking. Women provide the majority of caregiving in DLB but how this affects the caregiving experience is uncertain. Gaining a better understanding of sex differences will be instrumental in aiding future development of sex-specific strategies in DLB for early diagnosis, care, and drug development.