Merkel cell carcinoma (MCC) is an uncommon primarily dermal malignancy of relatively aggressive biologic course. Several presentations in the mucosa of the head and neck region have been reported in the literature, and 3 such patients have recently been seen at our institution. We review this recent experience and present the first reported primary lingual MCC in a 57-year-old caucasian man. We provide a review of oral mucosal MCC and guidelines for histopathologic and immunohistochemical diagnosis. Merkel cell carcinoma should be included in the differential diagnosis of head and neck mucosal lesions, especially if the tumor is submucosal, and MCC may involve the tongue. Mucosal MCC is aggressive, and there is a high risk for local recurrence and regional and distant metastasis. Fulminating courses are often seen. We discuss our treatment policies based on the current literature.