Huriez syndrome is a rare cancer-prone genodermatosis confined to the hands and feet connects with an increase in squamous cell carcinoma on affected skin. Its diagnosis is complex due to not well defined symptoms and since only few cases are described in literature. The differential diagnoses are many and the treatment is focused only on symptoms control and tumours eradication. Our case report is highly interesting because add new knowledge about this disease describing a new important feature of the syndrome. For the first time in literature we describe the arising of basal cell carcinoma from affected skin.