The patient with PV c.5487_5490dup in the NF1 gene developed bone, liver, and lung metastases at 22 years after PPGL resection and died after developing severe febrile neutropenia following therapy with 131I-mIBG; to our knowledge, this is the first NF1-associated PPGL case reported in which metastasis developed only 20 years later. This evidence concerns the gene NF1 and acquired polycythemia vera.