Ovarian cancer is currently the most fatal among gynecological malignancies with a low survival rate, especially in the case of type II tumors. Cancer antigen 125, the primary protein biomarker associated with ovarian cancer, has been researched for its involvement in the ovarian cancer pathogenesis and its potential applications in enhancing both accurate diagnostics and effective therapies. Research has investigated the connections between CA125 and several biological pathways, revealing their associations with cancer cell proliferation and migration. Further research aims to utilize CA125 for a more accurate diagnosis and potential treatments for ovarian cancer. Approaches have been established to address the low specificity of CA125 diagnostic tests, including altering the traditional CA125 value threshold used for ovarian cancer diagnosis and detecting CA125 alongside other antigens. CA125-specific cancer vaccines and immunotherapies are currently undergoing clinical trials and are being developed for future clinical use.