AbstractPrimary adenocarcinoma of the appendix is rare. Eleven patients with this rare neoplasm have been evaluated at our institution over a 50‐year period. We have reviewed the presentation and clinical course of these patients, and have compared them with those described in the literature. The presenting signs and symptoms, physical findings, and treatment were similar to those described in the literature. However, the 5‐year survival of 20% is lower than most series, and reflects the advanced stage of disease at the time of diagnosis in this group of patients. Despite the low 5‐year survival, we feel that aggressive therapy (right hemicolectomy) is necessary to obtain long‐term survival.