The aim of this study was to assess whether restorative proctocolectomy was suitable as an initial procedure for selected familial adenomatous polyposis patients with coexisting colorectal cancer. Six malignancy patients who underwent restorative proctocolectomy for familial adenomatous polyposis were reviewed. At the time of restorative proctocolectomy, cancer was not suspected in four patients. The two remaining familial adenomatous polyposis patients had a known associated colorectal cancer. Operative procedures, pathological findings, complications and long-term follow-up were evaluated. All patients were Stage I-II cancers. There was no mortality or pouch failure. No evidence of tumor recurrence was found and all the patients are still alive and disease-free in follow-up (mean 28 months). As an initial procedure, restorative proctocolectomy for patients with familial adenomatous polyposis with coexisting Stage I-II colorectal cancer seems to be a dependable procedure.