Due to histopathologic diagnosis, genetic testing for familial paraganglioma, neurofibromatosis type 1, von Hippel-Lindau disease, the Carney triad, multiple endocrine neoplasia type 2, and mutations of the succinate dehydrogenase genes (SDHB, SDHC, and SDHD) was performed that was negative. The gene discussed is SDHD; the disease is neurofibromatosis type 1.