A 10-year-old Brazilian girl presented with cutaneous lesions present since birth. She had been born to normal nonconsanguineous parents and was in good general health and of normal intelligence. Physical examination revealed dysmorphic facial features with frontal bossing. On the skin, there were innumerable tiny, follicular, filiform hyperkeratotic papules arranged in a band-like fashion following the lines of Blaschko. Some had coalesced into plaques. These isolated or confluent lesions were located on the right side of the back; right arm and axillary fold; and right side of the face, nose, brow, and scalp ( Figs. 1 and 2). Alopecia was observed on the affected area of the scalp. Intermingled with theses papules and plaques were several 0.5-to 1-cm nodulocystic lesions located mainly on the back, right arm, and scalp.Histopathologic aspects of a filiform hyperkeratotic lesion and of a nodule are depicted in Figs. 3 and 4.