Abstract An 81-year-old man developed a left lower lid lesion. The lid margin was red, thickened, and indurated with foci of ulceration and telangiectasia on the palpebral conjunctiva. The initial clinical impression was either a chalazion or sebaceous carcinoma, and an incisional biopsy was performed. By light and electron microscopy, the tumor demonstrated infiltrating cords of cells with an admixture of epidermoid and mucus-secreting elements arranged in a distinctive elongated tubular fashion. The involved lid was resected and reconstructed by a Hughes' tarso-conjunctival graft with marsupialization of the canaliculus. No recurrence has been encountered after a year. Mucoepidermoid carcinoma of the conjunctiva is a rare, locally aggressive neoplasm characterized in all seven previously reported cases by recurrence within 6 months, and a high incidence of intraocular or orbital invasion.