Acrokeratosis paraneoplastica (first described by Gougerot and Rupp in 1922) was named after Bazex who had then reported several cases in a French dermatological journal since 1965 (Bazex et al. in Bull Soc Fr Dermatol Syphiligr 72:182, 1965; Bazex and Griffiths in Br J Dermatol 102:301-306, 1980).METHOD: The study is a clinical case of a patient with acrokeratosis paraneoplastica.RESULTS: the patient was later diagnosed with a cervical lymph node metastasis and thereafter with a primary squamous cell carcinoma of the left upper lobe and upon treatment responded with the clearing of the skin changes.CONCLUSION: Identification of a paraneoplastic syndrome may enhance the earlier diagnosis of the associated tumor and may thus enable curative treatment.