Amyloidosis is a disease of unknown origin characterized by the widespread or localized deposition of amorphous hyaline material, amyloid, in various body tissues, including the ocular and orbital structures. Primary advanced localized amyloidosis involving the eyelid and conjunctiva is a rare cause of diffuse eyelid thickening. Management of these cases is often unsatisfactory because of the diffuse deposition of amyloid tissue and the increased risk of intraoperative hemorrhage, due to the fragile vessels encountered in amyloidosis. The possibility of recurrence also presents challenging treatment dilemmas. Therapeutic options include en bloc removal of the involved eyelid tissue, radiation therapy or, in advanced cases, no treatment. We describe an effective surgical approach that offers a safe, effective, and, most notably, repeatable procedure for the treatment of advanced ocular adnexal amyloidosis.