Objective: To report a rare case of multiple endocrine neoplasia type 1 (MEN 1) in conjunction with concomitant thymoma and thymic carcinoid.

Methods: We describe a never before reported case involving a 63-year-old female patient with MEN 1 who had synchronous thymoma and thymic carcinoid tumors. A review of the pertinent literature is also undertaken.

Results: Although prognosis is stage dependent for patients with thymoma, patients with thymic carcinoids and MEN 1 have been reported to have an extremely poor prognosis, with many patients dying of complications from thymic carcinoid rather than dying of other manifestations of MEN 1. Our patient underwent successful surgical treatment and remains under surveillance for all aspects of the MEN 1 syndrome.

Conclusions: Thymic tumors are rare, and thymic carcinoids, while very rare in occurrence overall, have a definite association with MEN 1. Thus, it is important for practitioners to screen for thymic tumors routinely in patients with MEN 1 and to treat such tumors aggressively when found because they can be a major cause of mortality. Many thymic carcinoids are far advanced before diagnosis, and optimal screening for and treatment of thymic carcinoid are still being developed.