An 8-year-old boy had a congenital, solitary verrucous lesion on his ear that histologically showed numerous maloriented infundibulocystic structures from which radiated immature sebaceous lobules as well as hair germs with rudimentary papillae. The surrounding stroma was fibrillary, containing large quantities of mucin. We conclude that perifollicular mucinosis, considered to be specific to Carney's syndrome, can also be observed in solitary folliculosebaceous hamartoma.