Juvenile xanthogranuloma (JXG) is a rare cutaneous disorder that predominantly occurs during infancy and early childhood. This is an uncommon benign disordercharacterized by solitary or multiple yellow-red papulonodules on the skin,and occasionally in other organs. It is one of the non-malignant, non-Langerhans cell histocytoses (non-LCH). Prognosis is excellent. The lesion requires no teatment and disappears spontaneusly in months to years. In this paper, a four-month-old girl who was admitted to our clinic with complaints of having two swallings on upper right arm over the skin and was diagnosed to have had JXG histopathologically by biopsy taken from the lesion was presented. The possible systemic organ involvement and the diseases which may accompanied were studied. JXG is limited to two lesions on the skin and no other organ involvements were determined. No treatment was performed and was followed. JXG, a rarely seen skin lesions, was reminded with this case.

Keywords: jüvenil ksantogranüloma, non-Langerhans hücreli histiyositozis, ksantoma, juvenile xanthogranuloma, non-Langerhans cell histiyositozis, xanthoma