Acrokeratosis paraneoplastica (Bazex syndrome) is a rare but distinctive dermatosis associated with carcinomas of the upper aerodigestive tract with possible cervical lymph node metastases. The cutaneous lesions occurring in the syndrome are non-metastatic skin involvement that parallels (as cutaneous marker) the evolution of the malignancy. Since only a few case reports have been published in the otolaryngologic literature, we present our experience and review some of the findings previously reported.