Juvenile xanthogranulomas (JXG) are benign, spontaneously regressing histiocytic tumors of unknown pathogenesis. The incidence is unknown, and there may be a slight male preponderance.

JXGs are usually solitary and most often appear at birth or within the first year of life. However, their number and size may increase over the first 18 months of life. Lesions usually regress over 3-6 years, leaving behind persistent pigmentary change or atrophy.

Systemic involvement is rare, with the most common site of extracutaneous involvement being the eye (0.3%-0.5%). Eye involvement is more common in patients with multiple JXGs. Many internal organs can potentially be involved, including the central nervous system, liver, lungs, muscles, oropharynx, and spleen. Cutaneous lesions are absent in one-half of cases with systemic involvement.

JXGs have been associated with neurofibromatosis type 1 (NF1), particularly in patients younger than 2 years of age. Additionally, a triple association between JXGs, NF1, and juvenile myelomonocytic leukemia has been observed in several case reports. In adults, the condition lasts longer and is more frequently associated with permanent changes in pigment and atrophy after spontaneous resolution.