The accurate distinction between primary and secondary mucinous ovarian cancers is a crucial tool for effective surgical and systematic treatment. Mucinous ovarian metastases of appendiceal origin are a special group of tumors because they appear even in half of female patients with primary appendiceal mucinous carcinomas and demonstrate pathological similarity to primary ovarian mucinous neoplasms. The current literature review focuses on the differences based on pre-operative symptoms, radiological findings, the spectrum of microscopic features, and the significance of the immunophenotype of each tumor. Treatment options, including surgical management and adjuvant chemotherapy protocols, are also briefly overviewed. In conclusion, the source of the ovarian tumor mass might be suggested by preoperative symptoms, values of antigens, and imaging findings. However, the confirmation of the tumor origin is only made after the postoperative pathological examination. Investigating the most accurate immunohistochemical markers and new molecular features may improve diagnostic efficiency in future research.