Goblet cell carcinoid of the appendix is a relatively uncommon clinical condition. Although its histogenesis remains controversial, its histological characteristics are distinctive enough not to pose a serious problem for diagnosis. The clinical behaviour of the tumor appears to lie between the typical behaviour of an ordinary carcinoid and that of a well differentiated adenocarcinoma of the appendix. Clinical diagnosis of this condition is seldom made pre-operatively, most patients presenting with signs and symptoms of an acute appendicitis. In a number of patients the tumor is identified incidentally during an operation performed for some unrelated entity. Treatment of this tumor is generally by simple appendectomy, although in some patients a more radical procedure is indicated. Prognosis for the most part is quite favorable with the exception of those few patients who present with the more virulent form of the disease.