To report a rare case of primary mucinous adenocarcinoma arising from a sweat gland in the eyelid.

A 68-year-old male presented to our hospital with a painless, superficial nodular lesion over the skin of the right lateral canthus that had slowly grown over the past two years. The patient had a history of surgical excision for three nodular lesions at the same site 5 years ago, and an excisional biopsy was mucinous adenocarcinoma with a positive margin. A systemic evaluation, including whole-body Positron Emission Tomography scan (PET), chest computerized tomography, gastrointestinal endoscopy, and colonoscopy, revealed no other abnormal lesions. Therefore, the eyelid lesion was considered a primary mucinous adenocarcinoma of the skin.

Primary mucinous adenocarcinoma of the eyelid can rarely metastasize. Therefore, a systemic examination is warranted to discriminate primary and metastatic adenocarcinoma and also to monitor the long-term follow-up for the evaluation of local recurrence.