Erdheim-Chester disease is a rare infiltrative disorder of non-Langerhans cell histiocytes, which affects multiple organs, including bone, heart, lungs, orbits, central nervous system (CNS), pituitary gland, and retroperitoneum. Bone affliction is almost universal in these patients and, to a lesser degree, the CNS, retroperitoneum, and other tissues. Eye involvement is typically retro-orbital or periorbital. This case report describes a 48-year-old patient who presented with reduced vision in the left eye and no other complaints. Fundus examination revealed a choroidal mass in the macula. The authors believe that this might be the second case of choroidal involvement in Erdheim-Chester disease.
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