Inherited renal cancer disorders that should be considered in patients with MPRT suspected of having a genetic cause but who test negative for a standard RCC gene panel test, include germline SDHx (SDHA, SDHC and SDHD if only SDHB has been tested), CHEK2 variants and two recently described RCC predisposition genes (ELOC and PRDM10) which are associated with syndromic forms of RCC. The gene discussed is SDHA; the disease is renal cell carcinoma.