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A rare case of mixed carcinoma of the ovary is reported, composed of a large cell neuroendocrine carcinoma and a mucinous borderline endocervical tumor. The large cell neuroendocrine carcinoma was composed of solid nests, sheets, and trabeculae of medium to large-sized cells, and was positive for synaptophysin. The mucinous epithelial tumor varied in appearance from a borderline to an intraepithelial carcinoma, and showed sparsely scattered immunoreactivity for chromogranin-A. Using an X-chromosome clonality assay, these 2 components showed patterns of monoclonality. These results suggest that the large cell neuroendocrine carcinoma may have arisen from the mucinous epithelial tumor.