An 82-year-old woman had a dark red to purple tumor on the left buttock that had gradually enlarged during the last 5 years. Although routine histologic examination was not sufficient for diagnosis, neuroendocrine carcinoma was diagnosed by immunohistochemical and ultrastructural studies. Immunohistochemical-positive reactions to neurofilament, cytokeratin, neuron-specific enolase, and epithelial membrane antigen were noted. Electron microscopically, membrane-bound, dense core granules that yielded a positive uranaffin reaction and intermediate filaments in the perinuclear area were observed in the cytoplasm of most tumor cells. Desmosome-like structure between them was also found. Approximately 6 months after local excision, metastatic lesions developed in the regional lymph nodes and liver.