We report the observation of a 24-year-old woman presenting a plaque-like lesion of the occipital scalp with hair rarefaction. This lesion was congenital and consisted of numerous, agminated, very small, trichilemmal cysts. It remained stable since birth, slowly growing in proportion with the patient during childhood. We discuss the nosological status of this intriguing lesion and its relationship with the recently described trichilemmal cyst nevus.