Because of the presence of infiltrating group of signet ring cells in mucin producing tumor of vermiform appendix, this tumor has often been diagnosed and treated as adenocarcinoma. However, it was recently recognized that this tumor is histologically and biologically similar to a carcinoid tumor. In view of the clinically and morphologically benign course of this tumor, radical surgery beyond appendectomy is questioned. A case of unusual mucoid variant of carcinoid tumor of appendix in a 52 years old male is presented with review of the literature.