The clinical and pathological features of warty dyskeratoma are presented. Warty dyskeratoma is a rarely encountered, solitary, benign cutaneous tumor occurring most often on the head and neck and to our knowledge, no cases associated with cutaneous horn have been published in western literature. We experienced a case of warty dyskeratoma with a cutaneous horn occurring in a 70-year-old male, who had a 1.3 cm-sized and slowly growing nodule on his neck. A brief review of the literature, was made especially in relation to the pathological findings and histogenesis of warty dyskeratoma.