We experienced a case of argyrophil carcinoid of the appendix in 36-year-old woman who was admitted to the hospital because of lower abdominal pain, nausea and vomitting. We performed appendectomy next day under the diagnosis of acute appendicitis. Resected appendix was whitish, hard and swollen, and histological examination revealed proliferation of tumor cells mainly in the submucosal layer of the appendix. The tumor cells were positive with Grimelius and chromogranin A stain, and negative with Fontana-Masson stain. Therefore, we diagnosed the case as argyrophil carcinoid of the appendix. Because of the positive surgical margin, ileocecal resection with lymphadenectomy was performed 55 days after the initial operation. There were no tumor cells microscopically in the resected specimen. There is no signs of recurrene in one year and 4 months since the operation. To our knowledge, argyrophil carcinoid of the appendix is very rare in Japan. Here, we report this case with a review of the literature.