Acrokeratoelastoidosis of Costa is a rare papular palmoplantar keratosis characterized by small round-oval to rhomboid-shaped, yellowish papules most commonly localized to the palmar surface of the hands and sometimes also found on plantar surfaces of the feet. Both autosomal dominant and sporadic forms of the disease have been reported. Histologically acrokeratoelastoidosis is marked by hyperkeratosis and epidermal hypertrophy. Specific stains for elastic tissue reveal characteristic fragmentation and rarefaction of elastic fibers. We report a sporadic case of acrokeratoelastoidosis in a 13-year-old girl and review the etiology, differential diagnosis, and treatment of this disorder.