Familial sebaceous gland hyperplasia is a benign entity with onset at puberty and a tendency to worsen with age. It is characterized by a nevoid symmetric pattern in the sebaceous areas of the face sparing the orbital, perinasal, preauricular and perioral areas showing prominent follicular openings and interfollicular dermal yellowish or white prominent skin. Usually, the face is affected, with the neck, column and thorax affected in a milder pattern. We describe a family with familial nevoid sebaceous gland hyperplasia in three consecutive generations. All the patients were successfully treated with oral isotretinoin. The pedigree suggests autosomal dominant inheritance with incomplete penetrance.