Up to 40% of cases may be associated with an inherited familial syndrome, including succinate dehydrogenase (SDH) enzyme mutations.[3] Any mutation of the SDH complex subunit (SDHB, SDHC, SDHD, SDHAF2, and SDHA) predisposes mainly to the development of PPGL, but the recent literature also reported other SDH-related lesions such as gastrointestinal stromal tumors (GIST), renal cell carcinomas, and pituitary adenomas (PA).[4,5] We describe here the case of a 31-year-old patient who presented the uncommon association of paragangliomas and pituitary adenoma. Here, SDHB is linked to renal cell carcinoma.