ConclusionThis case illustrates several points frequently emphasized in medical literature: 1) A person who has malignant disease must be watched carefully for subsequent development of metastasis and other primary lesions. 2) Multiple polyposis must be treated aggressively and as soon as possible. 3) Prognosis, even in an apparently hopeless situation, should be guarded. 4) A patient with known biologic resistance to malignancy should be given the benefit of standard surgical procedures in the management of new neoplasms that arise subsequent to the appearance of the initial carcinoma.We have presented a report on a male patient having congenital multiple familial polyposis with associated multiple primary malignancy of the colon, periampullary carcinoma of the ampulla of Vater, and a third primary lesion — a transitional‐cell carcinoma—of the urinary bladder. His over‐all survival time, from the appearance of the initial carcinoma of the rectum to the present, is 13 years. During this time there was spontaneous regression of pulmonary metastases during an interval period of six years.