Familial adenomatous polyposis (FA ) is a hereditary disease with autosomal dominant transmis-sion, it is a practically 100 % precancerosis. For detection of further patients in the family careful exanmination of the patient and all subjects at risk, i.e. abve all grade 1 relatives, is decisive. The presented paper summarizes the author´s own experience with the follow up and examinati-on of a group of 96 patients from 42 families. The group of patients has been assembled gradually since 1967. The basis of the examination is preparation of a pedigree, somatic examination, endoscopic exa-mination of the large intestine and the oral portion of the digestive tract. Examination of the ocular fundus is valuable as it evaluates the presence of congenital hypertrophy of the retinal pigmented epithelium (CHR E). A positive finding in relatives permits conclusions on the presen-ce of FA . Most evidence is provided by molecular genetic examination at the DNA level which makes presymptomatic diagnosis of FA possible.

        Key words: Familial adenomatous polyposis - Congenital hypertrophy of the retinal pigmented epithelium - Presymptomatic DNA diagnosis of FAP