The carcinoid tumor of the middle ear is a very rare neoplasm which in general is regarded as benign but may be mistaken for an adenocarcinoma because of its histological heterogeneity. Typical, however, is its neuroendocrine and mucinous differentiation so that an unequivocal diagnosis is possible by means of immunohistochemistry and electron microscopy. As the tumor is very rare, there is no statistical evidence as to whether further treatment is necessary after primary exstirpation of the tumor. Therefore, a review of the literature was performed. We report about a 28-year-old male patient with a carcinoid tumor of the left tympanic cavity. Without any further treatment there has been no evidence for either recurrence or metastases 32 months after surgical resection. As treatment of choice we recommend conservative surgery with complete removal of the tumor and a clinical follow-up on a regular basis.