AbstractPrimary mucinous adenocarcinoma of the skin is a rare entity arising from eccrine sweat glands with a propensity for local recurrence and metastasis. This tumor has been widely reported in the ophthalmology literature, but not to our knowledge in the otolaryngology literature. A case of a 48‐year‐old man with a mucinous adenocarcinoma extensively involving the right eyelid and orbit necessitating craniofacial resection is presented together with a discussion of the pathology, clinical features, and management. © John Wiley & Sons, Inc.