A carcinoid tumor of the thymus combined with thymoma in a 62-year-old man is described. The mediastinal tumor had been present for 13 years and was associated with pure red cell aplasia. Carcinoid tumor occupied the central two-thirds of the tumor, consisting of nests and trabeculae of monotonous round cells, which ultrastructurally showed many intracytoplasmic dense-core granules. Typical spindle cell type thymoma surrounded the carcinoid area. Clinico-pathologic findings of this unique case suggested that the carcinoid tumor developed within a preexisting thymoma, illustrating a possibility of neuroendocrine differentiation of thymic epithelial cells.