TEMPI (telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting) syndrome is a recently described rare plasma cell neoplasm with paraneoplastic features.1,2 In this case report, we describe a patient with TEMPI syndrome with classic and atypical cutaneous findings that track with the progression of pulmonary symptoms.