Acanthosis nigricans (AN) is a disorder often accompanied by internal malignancy in adult patients, but in children it is usually benign. Generalized forms of AN, especially in childhood, are extremely rare. We report a 5-year-old boy with a 3-year history of AN who had generalized roughness and hyperpigmentation, numerous skin wrinkles, disseminated skin tags, tripe palms, and intensive pruritus. No clinical evidence of internal disorders, notably of an endocrinologic or neoplastic nature, have been detected thus far.