&NA;Clinicopathologic correlation and survival were evaluated in 11 patients with adenocarcinomas of the appendix. This extremely rare tumor was seen most often in patients in the fifth decade of life. Acute appendicitis was the most common mode of presentation (8/11). A few patients (3/11) showed signs of distant metastases from an occult primary tumor in the appendix.Overall, median survival and five‐year survival rate were two and a half years and 36 per cent, respectively. The patients who had papillary adenocarcinoma had the best prognosis because the tumor manifested early in the form of appendicitis. Those who had the colonic type of adenocarcinoma had a poorer prognosis because of full‐thickness invasion (8/9) and lymphnode and distant metastases (5/9) at the time of diagnosis.Perforation of the appendix,per se, does not imply poor prognosis, but when it is at the site of tumor, the prognosis is worse. Every appendix should be opened immediately after its removal. Right hemicolectomy should be performed either at the time of the first operation or following initial appendectomy.