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The paper presents a clinical case of appendiceal mucinous adenocarcinoma with invasion into the right ovary, imitating an ovarian tumor. The primary tumor was diagnosed only after a pathological evaluation of the surgical material with differential immunohistochemical assay after laparotomy and removal of the ovarian tumor and appendectomy. Difficulties in establishing an accurate diagnosis were caused by extensive invasion of the appendiceal tumor into the ovary. This disease requires increased oncological alertness and a clear algorithm for differential diagnosis of mucinous tumors of the appendix and ovary.