Juvenile xanthogranuloma is a non-Langerhans cell lesion mostly limited to the skin but occasionally presenting in extracutaneous locations or associated with systemic conditions. Lesions need to be distinguished mainly from dermatofibroma, xanthoma, Langerhans cell histiocytosis, or reticulohistiocytoma. Herein, we present a hemosiderotic variant of juvenile xanthogranuloma in a 12-year-old girl, which we have not found described in literature. The lesion presented at the back of the scalp as a slowly growing yellowish polypoid lesion showing occasional bleeding. The histopathological examination demonstrated a cellular infiltrate expanding the dermis, with a Grenz zone and with no remarkable changes in the overlying epidermis. The papule was made of mononucleated macrophages, many of which were xanthomatous. There were some Touton giant cells. The lesion was intermingled with a mild inflammatory infiltrate comprising lymphocytes, plasma cells, neutrophils, and some eosinophils. Many of the macrophages contained abundant cytoplasmic deposits of iron. The macrophages expressed CD68 and CD163, whereas they failed to express S100 protein, CD1a, and Langerin.