A 63-year-old man, in good health previously, developed swelling and erythema of the right upper eyelid which progressed to involve both eyelids and surrounding tissues. After one month, he had a 9.0 x 9.0 x 3.5 cm mass despite two attempts at incisional drainage and treatment with antibiotics. Multiple asymptomatic erythematosus nodules (2-3 cm in diameter) developed on his back, trunk, and arms four weeks after onset of the eyelid erythema and swelling. Biopsies of two upper back papules and the eyelid disclosed cutaneous T-cell lymphoma, a lymphoproliferative malignancy of thymus derived lymphocytes originating in the skin. Systemic chemotherapy resulted in a marked decrease in the ocular adnexal mass but lymphomatous meningitis and quadriplegia developed. The patient died four months later; an autopsy was not performed. This case demonstrates that cutaneous T-cell lymphoma may rarely have its initial manifestation as a rapidly enlarging eyelid tumor.