Although metabolic diseases manifesting with cutaneous findings are rarely seen in daily practice, they emerge clinical significance as they can be the first manifestation of the systemic disease or can be seen in conjunction with other disorders. Among these diseases, scleromyxedema, diffuse normolipemic plane xanthoma, necrobiotic xanthogranuloma, amyloidosis and porphyria cutanea tarda can accompany myeloproliferative and lymphoproliferative diseases, as well as the malignancies of the solid organs. This paraneoplastic relationship can be diagnosed simultaneously at the time of the diagnosis of the cutaneous disease, or the cutaneous disease can precede the malignancy by several years. The recognition of these cutaneous diseases, appropriate screenings for the associated malignancies and the long term follow up of the patients can help to reduce the morbidity and mortality. Furthermore the treatment of the cutaneous disease can solely be possible with the management of the associated neoplasm.