Colorectal cancer in familial adenomatous polyposis is a preventable disease in at-risk relatives of patients with primary cases. Until the recent establishment of a register in Western Australia, there has been no registration of pedigrees or central organization of surveillance in Australia. In the present study, the experience of 20 such families who were associated with The Royal Melbourne Hospital was documented, with an analysis of the reasons for any failure of management. The impact of a hospital-based register on the management of the disease was studied. In each family, results were categorized according to whether “at-risk” relatives had been diagnosed at surveillance examinations, and whether the Hospital register were involved. Before involvement with the register, 24 family members presented with symptomatic polyposis after the first affected case had been diagnosed. Eighteen of these had colorectal cancer at diagnosis, and 16 subjects now are dead. Identifiable reasons for the failure of surveillance were family communication failure (two cases), family denial (two cases), failure of the hospital clinic (two cases) and a failure to cover extended branches of families who were living locally (nine cases), interstate (four cases) or overseas (three cases). In contrast, only two (6%) of 33 affected cases that were identified at a planned surveillance endoscopy had colorectal cancer at diagnosis. Without the active surveillance of at-risk family members, lethal delays in diagnosis are likely to occur. Most reasons for failure potentially are correctable by a dedicated registry that is responsible for notifying clinicians and patients about the timing of surveillance procedures.