We describe a rare case of glomus tumor of the duodenum. The tumor was detected in a 46-year-old man being investigated for episodes of gastrointestinal bleeding. The excised specimen showed a 2.3 x 1.5 x 1.5 cm gray-tan tumor involving the mucosa and the submucosa. The overlying mucosa was focally ulcerated. Histologically, the tumor had a solid pattern of sharply demarcated, round glomus cells with prominent pericytoma-like vessels of varying sizes. The neoplastic cells stained for vimentin, smooth muscle actin, and focally for synaptophysin. These histologic and immunohistochemical features are consistent with a diagnosis of glomus tumor of the duodenum.