A rare case of mixed carcinoma of ovary with a predominant malignant neuroendocrine and a lesser mucinous tumor component is described. Extensive areas of enteric-type epithelium containing endocrine cells were present in both the mucinous borderline tumor element and in the small foci of mucinous adenocarcinoma. Transition from the mucinous tumor to the malignant neuroendocrine component provides evidence for the derivation of some neuroendocrine or carcinoid tumors direct from mucinous tumors without necessarily being a component of a teratoma. Contrary to reported examples of mucinous carcinoid, the overgrowth and spread of the neuroendocrine component characterised the aggressive and lethal nature of this tumor.