A case of high-grade endometrioid adenosquamous carcinoma of the ovary showing pilomatricoma-like areas and neuroendocrine differentiation is presented. The pilomatricoma-like areas were strongly positive for cytokeratin and vimentin, whereas the poorly differentiated areas of the tumor were positive for cytokeratin, synaptophysin, and neuron-specific enolase. Review of the literature did not show any published report of adenosquamous carcinoma with pilomatricoma-like areas either in the ovary or in the uterus.