Paragangliomas may be hereditary and can be associated with 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) [1]. Here, SDHD is linked to paraganglioma.