To report a case of adult orbital xanthogranulomatous disease presented as bilateral swelling and yellowish eyelids in a 42-year-old woman who was misdiagnosed with xanthelasma at a dermatologic clinic.

A 42-year-old woman presented yellowish and swollen eyelids in both eyes of 2.5 year duration. She had no past history of systemic diseases or other ophthalmologic problems. MRI showed heterogeneous eyelid masses and hypertrophic changes of the lacrimal glands in both eyes. There were no abnormalities on chest X-ray exams. The blood cholesterol and low density lipoprotein levels were increased. Incisional biopsy showed many foamy histiocytes, lymphocytes with germinal centers, several touton giant cells, and negative S100/CD 1 staining; all being features consistent with adult onset xanthogranulomatous disease.

In cases with bilateral swelling and yellowish colored eyelids, adult orbital xanthogranulomatous disease should be evaluated through biopsy and other systemic examinations.