Endocrine mucin-producing sweat gland carcinoma is a rare skin tumor that most commonly involves the eyelid of elderly women. Morphologically and immunohistochemically, it is analogous to endocrine ductal carcinoma in situ of the breast and mammary solid papillary carcinoma; also, like the analogous breast lesion, there is an often associated invasive mucinous carcinoma with neuroendocrine differentiation. We describe the case of a 65-year-old woman with endocrine mucin-producing sweat gland carcinoma of the eyelid that recurred 3 years after an apparently complete excision.