Ovarian clear cell cancers (OCCC) represent 5–25% of ovarian cancers. Histologic diagnosis remains a significant challenge, often misclassifying the cancers. These tumours tend to present at earlier stages, and their aetiology is linked to endometriosis and genetic aberrations such as ARID1A and PIK3CA mutations. When compared, stage for the stage with other epithelial ovarian cancers, patients with early-stage OCCC have a better prognosis than patients with high-grade serous tumours. Patients with Stage IC–IV have a relatively poor prognosis, and efforts should centre on discovering more effective treatment strategies. Ovarian CCC is a biologically distinct entity, different from high-grade serous epithelial ovarian cancers (EOC). Future research is needed to explore targeted therapy’s role in managing ovarian CCC.
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