A 3-year-old boy had maculopapules on his face and neck since age 6 months. These were yellow-brown, asymptomatic, and clinically similar to flat warts. Histopathologic study revealed a fibrohistiocytic infiltrate in the superficial dermis. Ultrastructurally, comma-shaped bodies, desmosome-like junctions, and coated vesicles were seen; there were no lipid droplets or Birbeck granules. With these data, a diagnosis of benign cephalic histiocytosis was made. Twenty-five cases are reported in the literature: 17 males and 8 females (male:female ratio 2:1). Sixteen patients had lesions on parts of the body other than the head, neck, and shoulders.