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Mucinous ovarian cancer (MOC) is a rare pathological type different from epithelial ovarian cancer, and the clinical treatment should refer to serous ovarian cancer (SOC) guidelines. However, since the clinicopathological features of MOC are significantly different from SOC, careful differentiation is needed in diagnosis and treatment. Surgery combined with adjuvant chemotherapy is the standard treatment for MOC. However, due to the low prevalence rate, it is difficult to carry out clinical trials, hence lacking evidence-based medicine and consensus on the indications of intraoperative appendectomy and the choice of postoperative adjuvant chemotherapy. In addition, further translational preclinical studies of targeted therapy and immunotherapy are needed to facilitate the diagnosis and individualized treatment of MOC.