Perifollicular fibroma (PFF) is a rare cutaneous hamartoma that shows differentiation to the connective tissue sheath of the hair follicle. It may be solitary or multiple; the latter has been suggested to exhibit autosomal dominant inheritance and association with colonic polyposis. We report a 72-year-old Japanese female with multiple PFF on the face, possibly familial, who developed colon cancer in addition to colonic polyps.