Amyloidosis involving the eyelid is a rare condition. We report a case of primary systemic amyloidosis of the eyelid.

A 26-year-old female presented with multiple nodules on the bilateral upper and lower eyelids that had stopped growing several years prior. Multiple pearl-colored small nodular lesions were present on the upper and lower eyelid bilaterally and no clinically specific signs were observed. Surgical excision, biopsy and electrocauterization were performed. Histological examination showed amorphous and eosinophilic substances on hematoxylin & eosin (H&E) staining and orange-colored amyloid deposits stained with Congo-red. Systemic evaluation showed amyloid nodules invading the vocal cords and external auditory canal, therefore the patient was diagnosed with primary systemic amylodosis. At the postoperative 6-month follow-up, recurrence or inflammation at the operation site was not observed.

To the best of our knowledge, this is the first case of primary systemic amyloidosis of the eyelid in Korea. Amyloidosis should be considered in a differential diagnosis of a mass in the eyelid and can be successfully managed with surgical excision and electrocauterization.