We report seven cases of adenocarcinoma of the vermiform appendix occurring in Iceland during 1974–1989. The patients ranged in age from 25–83 years, mean age 55.1 years. There were five males and two females. Five had mucinous adenocarcinoma, two had adenocarcinoma. Four patients presented with symptoms and signs of acute appendicitis and all had surgically resectable disease. Three of these patients were alive with no evidence of disease four months, two years and 15 years after presentation; one death of disease occurred seven years after ileocecal resection. In three cases, the clinical presentation was that of metastatic adenocarcinoma of unknown origin. Of these patients two were diagnosed at autopsy and one after appendectomy for perforated appendicitis. Survival in this group was six weeks, three months and twelve months, respectively. In none of our patients was the diagnosis made preoperatively and no tumors were found in appendices removed incidental to other intra‐abdominal operations. The incidence of adenocarcinoma of the vermiform appendix in Iceland during 1974–1989 was approximately 0.2 cases/100.000/year.