Juvenile xanthogranuloma is a congenital or perinatal tumor, 1 to 2 cm in diameter, usually located on the head. The extracutaneous lesions can occur on the eye, the lung, the epicardium, the oral cavity or the testicles. Subcutaneous form of juvenile xanthogranuloma has been reported very rarely in the literature. We report a unique case of a subcutaneous juvenile xanthogranuloma that showed 4 X 4 cm sized plaque and located on the extremity of 9-year-old girl.