We report a patient with keratosis punctata palmaris et plantaris who developed adenocarcinoma of the colon. Family history revealed at least seven other individuals with punctate keratoderma. The patient's mother had punctate keratoderma and carcinoma of the colon, and his maternal grandfather had punctate keratoderma and carcinoma of the pancreas. This is the third reported family, to our knowledge, in which hereditary palmar and plantar hyperkeratosis appears to be associated with gastrointestinal carcinomas.