Neurofibromatosis type 1 (NF1) is an autosomal dominant, neurocutaneous disease affecting mainly skin and central nervous system. Café au lait spots and axillary/inguinal freckling are most common skin manifestations. Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis which is characterized by yellow-orange colored nodular skin lesions. JXG has been rarely reported together. Skin manifestations of NF1 persist life long however JGX subsides spontaneously. Optic glioma is the most common tumor with NF1 patients. Coexistence of NF1 and JXG increases the risk of leukemia. We report two boys with JXG and NF-1 in this article. Copyright © 2011 by Türkiye Klinikleri.