This summary highlights the significance of Acrokeratosis neoplastica (Bazex syndrome), a skin condition characterized by erythematous hyperkeratosis with yellowish scales, primarily on acral locations. The syndrome is notably associated with a high risk of malignant diseases, particularly squamous cell carcinomas (SCC) of the glottis and lung. The study underscores the importance of recognizing Bazex syndrome as a potential diagnostic indicator for hidden malignancies, emphasizing its role in facilitating early cancer detection and treatment. Additionally, the review expands on the clinical manifestations of Bazex syndrome and provides updated insights into its pathogenesis. This information is crucial for medical professionals, as it reinforces the need for vigilance in patients presenting with specific dermatological signs, potentially leading to timely diagnosis and management of associated malignancies.