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. Here, SDHB is linked to neurofibromatosis type 1.