AimThe increasing incidence of cesarean sections has led to a higher prevalence of cesarean scar defects (CSDs), predominantly characterized by postmenstrual bleeding in affected women. CSD manifests in various forms, including isolated myometrial defects, intrauterine cystic protrusions, and extrauterine cystic extensions. The pathophysiological mechanisms underpinning CSD and its associated symptoms remain insufficiently understood. This review explores the pathogenesis of CSD, highlights its morphological characteristics with an emphasis on variable clinical diversity, and addresses the challenges for future research.MethodsA comprehensive narrative review was conducted using electronic databases, including PubMed and Google Scholar, to identify 41 relevant literatures published up to December 31, 2023.ResultsThe myometrium comprises two distinct layers—the inner and outer myometrium—with differences in their origin, structure, and function. Disruptions within these layers contribute to CSD development. Histopathologically, symptomatic CSD may be linked to uterine scar endometriosis, cystic adenomyosis, or endometrial cysts, suggesting that CSD, particularly those necessitating surgical intervention, are not limited to myometrial defects but may also involve iatrogenic endometriosis or adenomyosis, thereby exacerbating clinical symptoms.ConclusionThis review provides an updated understanding of the histopathological features and classification of CSD, with an emphasis on elucidating its underlying pathogenesis.