Introduction: Juvenile xanthogranuloma is a non-Langer hansian histiocytosis. In 5 to 17% of cases the lesions are present at birth. We report a new case of congenital juvenile xanthogranuloma.
Case report: A four-month-old female infant with no significant pathological history had a single nodular lesion 1 cm in diameter at the left knee flexion surface gradually increasing in size, orange-yellow in color, with squamous surface, with regular contours, firm and painless, with no extracutaneous signs or associated signs of Neurofibromatosis type 1.
Histological examination found juvenile xanthogranuloma with a dermis containing dilated lymphatic ducts with a foamy, histiocytic interstitial infiltrate and hyperplastic epidermis.
Discussion: It is a benign cutaneous lesion of infants and young children. The cephalic extremity and the nape of the neck are preferentially affected, but the lesions can also occur on the trunk and the extension faces of the extremities. The evolution is generally favorable and the lesion regresses spontaneously in almost all cases.
Conclusion: Juvenile xanthogranuloma is a benign cutaneous tumor. Nevertheless, cases of fatal evolution are reported in multiple locations from where the interest of a careful clinical examination with search for signs of extra-cutaneous appeal.