Acrokeratosis paraneoplastica of Bazex is rare condition, characterized with scaly hyperkeratotic psoriasiform plaques on acral parts of body (helices, nose, and malar and acral surfaces), and in later stages propagation to the limbs and trunk.This syndrome is distinct marker for different neoplastic conditions, predominantly squamous cell carcinoma of the upper aerodigestive tract with possible cervical lymph node metastases.In this paper we present 56 years old male patient, with hyperkeratotic plaques on the skin of his palms, soles, ear lobes and apex of the nose. Detailed examination found tumorous swelling on the left side of his neck. Histopathologic examination revealed solid anaplastic metastatic tumor. Patient died before primary tumor could be found. Bazex syndrome can appear before the diagnosis of internal malignancies, and thus is important for dermatologists to recognize it in favor of early diagnosis of specific malignant process.