Abstract: A 2-year-old boy had hundreds of discrete, small, flat yellow to brown colored papules distributed over the face, neck, and trunk. Biopsy revealed a well-demarcated infiltrate within the papillary dermis composed of histiocytes and some Touton giant cells and eosinophils. The cells were negative for protein S-100 and CD1a but positive for CD68 staining. No systemic lesions of juvenile xanthogranuloma were identified. Lichenoid juvenile xanthogranuloma is a very infrequent variant of juvenile xanthogranuloma.