The most frequent syndromes associated with multiple renal tumors are Von Hippel-Lindau (VHL), hereditary papillary renal cell carcinoma (HPRC), tuberous sclerosis complex (TSC), hereditary leiomyomatosis RCC (HLRCC), succinate dehydrogenase B deficiency (SDHB), and Cowden syndromes. The gene discussed is SDHB; the disease is hereditary leiomyomatosis and renal cell cancer.