A case of thymic atypical carcinoid with Cushing's syndrome and unfavorable clinical course is reported. Immunohistochemical analysis reveals distinct staining of tumor cells for ACTH, neuron-specific enolase, chromogranins (CG) and S-100 protein and with PHE-5 monoclonal antibody. At an ultrastructural level, the cells are undifferentiated with only a few neurosecretory granules. In the present case, immunohistochemical stainings for CG and with PHE-5 antibody seem reliable diagnostic tools, easily demonstrating the neuroendocrine nature of the neoplasm. NSE immunoreactivity can be an additional criterion. S-100-positive cells, which are present throughout the tumor, recall 'sustentacular cells', described in other neuroendocrine tumors.