Familial adenomatosis coli (FAC) traditionally has been diagnosed as the presence of more than 100 colonic adenomas, even if no familial occurrence is present. In the present communication, clinical features of FAC have been compared in detail to detect differences between familial and nonfamilial cases with regard to colonic and extracolonic lesions and to discover whether they constitute the same disease entities. No significant difference was found in the incidence of extracolonic lesions. The average number of colonic polyps in the nonfamilial cases of patients over 30 years old was 1128, the number of polyps was significantly higher in the familial cases (3154). When three patients with approximately 100 polyps were excluded, the mean number of colonic polyps in nonfamilial cases became 2608. This was similar to that of familial cases. Furthermore, the occurrence of approximately 100 polyps was extremely rare in familial patients who were over 30 years old. Therefore, most of the nonfamilial cases can be considered to be familial probands of FAC, but some, such as the three cases in the present study, may be a different disease entity, such as recessive adenomatous polyposis or multiple colonic adenomas.