Juvenile xanthogranuloma masquerading as melanoma

Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. We present an atypical case of bilateral JXG involving the entire uveal tract that presented with an aggressive phenotype. The patient was unresponsive to topical and systemic corticosteroids, cyclosporine, and maximal doses of radiation therapy. The disease was ultimately controlled with the alkylating agent chlorambucil.