Carcinoma of the appendix vermiformis is a very rare entity. In this paper the case of an appendiceal cancer in a 67-year-old woman is described. The patient showed a polyp in the caecum and underwent appendectomy and caecum excision. Histological examination revealed villous adenoma of the caecum as well as adenocarcinoma of the appendix. 14 days later right hemicolectomy was performed. The patient underwent adjuvant chemotherapy. In the discussion the frequency and prevalence of appendiceal carcinoma as well as its clinical manifestation, prognosis, diagnostic and treatment possibilities are reviewed.