Malignant neoplasms may present as paraneoplastic syndromes with mucocutaneous manifestations, which may or may not be chronologically associated. The pathophysiological mechanism is complex and not completely understood; therefore, definitive diagnosis may be achieved with a precise differential diagnosis based on the morphology of skin lesions, clinical picture, and histological pattern. The complexities, and low frequency, make the therapeutic approach quite challenging; consequently, the cornerstone of therapy is the eradication of the underlying neoplasms. Corticosteroids are the therapy of choice for most of these immune-mediated manifestations, but for the most part, the successful resolution requires the eradication of the underlying malignancy.