The skin and subcutaneous tissue and mucosae of the head and neck are areas of predilection for granular cell tumors. These often histogenetically enigmatic lesions have two principal clinicopathologic presentations: an infantile (congenital) epulis form of the gnathic gingiva and a noncongenital soft tissue and aerodigestive form. Recurrences of either form are largely attributable to marginal excision and, for all sites, approach 15%. A malignant granular cell tumor is recognized, but requires rigorous histologic confirmation.