Epilepsy affects people of all ages and sexes. The aim of the current narrative review was to summarize the sex differences in patients with epilepsy. In January 2024, we systematically searched PubMed for relevant articles. The following keywords were used: “Epilepsy” or “Seizure” and “Sex”. The prevalence and symptomatology of many neurological conditions, including epilepsy syndromes, may differ between male and female patients. The reasons behind these sex differences are not yet clear. It is likely that sex hormones, neurosteroid, and sex chromosome gene differences play roles in the development of this phenomenon. The existence of sex differences in epilepsy is well recognized, but there is little discussion of their mechanisms and therapeutic implications. Future research should investigate the exact roles of sex hormones, neurosteroid, and genes in the development of sex differences in epilepsy. Similarly, future studies should investigate whether sex differences exist in seizure characteristics (e.g., seizure frequency, semiology, response to treatment, outcome) in different syndromes. These issues may have important clinical implications for designing appropriate sex-specific treatment strategies for various syndromes and conditions. Furthermore, sex should be considered as a biological variable in basic and clinical research.