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Juvenile xanthogranuloma (JXG) is a rare, benign, self-healing tumor of unknown etiology arising from non-Langerhansâ€™ cell histiocytes. We report a case of 2 year old female child with few nodular lesions mainly over scalp, face, trunk and upper limbs. There was no other organ involvement. Histopathology showed presence of vacuolated histiocytes with Touton giant cells. There was concurrent hydrocephalus.