Adult xanthogranulomatous disease of the orbit including adult-onset asthma and periocular xanthogranuloma is rare. To the best of the author's knowledge, adult-onset asthma and periocular xanthogranuloma have not been previously reported in Korea.

A 75-year-old man presented with a 15-year history of progressive bilateral yellowish eyelid mass. He had a history of adult-onset asthma diagnosed 13 years ago. On ocular examination, there were firm yellowish bilateral eyelid masses and ptosis markedly on the right eye. Exophthalmos was noted in the right eye. There were exotropia, hypertropia, and gaze restriction of the right eye. Orbit magnetic resonance imaging demonstrated an ill-defined bilateral infiltrative orbital mass extending to the lid, lacrimal gland, and sinuses. A right orbital mass extended to the posterior orbit. Enlargement of extraocular muscles was also noted in the right eye. Left upper eyelid mass debulking surgery was performed. A combination therapy of azathioprine and prednisolone was started. Mass debulking surgeries of the right upper and lower eyelids were performed.

Adult-onset asthma and periocular xanthogranuloma is known as a refractory disease. Combination therapy with azathioprine and steroid therapy with mass debulking surgery is a good treatment option for adult-onset asthma and periocular xanthogranuloma.