In the two cases of G2 pNET with probable PTHrP-related hypercalemia we observed: in the first case, the normalization of calcium levels after therapy with Denosumab and PRRT; in the second case, patient died due to worsening of his performance status despite the different lines of treatment used to achieve disease stability (SSAs, Everolimus, PRRT) and to control hypercalcemia (hydration, loop diuretics, Zoledronate, corticosteroids, Denosumab). This evidence concerns the gene PTHLH and hypercalcemia disease.