First described in 2011, the TEMPI acronym denotes (1) telangiectasias, (2) elevated erythropoietin and erythrocytosis, (3) monoclonal gammopathy, (4) perinephric fluid collections, and (5) intrapulmonary shunting, that are the hallmark of this very rare paraneoplastic syndrome [2, 3]. The gene discussed is EPO; the disease is monoclonal gammopathy.