The hereditary renal cancers that have been identified include: ①VHL syndrome; ②Hereditary renal papillary adenocarcinoma; ③Hereditary smooth muscle tumor disease renal cancer; ④BHD (Birt-Hogg-Dube) syndrome. (a) Diagnostic points of hereditary renal cancer: ① the age of disease is mostly middle-aged and young, with/without family history; ② renal tumors are often bilateral and multiple, with imaging features of renal cancer; ③ other manifestations of the above syndromes, such as VHL syndrome may be combined with changes of central nervous system and retinal angioblastoma, pancreatic cyst or tumor, adrenal pheochromocytoma, epididymal papillary cystadenoma, renal cyst, etc. ; ④Testing to confirm the corresponding chromosomal and genetic abnormalities. (II) Treatment of hereditary renal cancer VHL syndrome is more frequently reported, while other types of hereditary renal cancer are only reported in case reports or small sample of cases. Most hereditary renal cancers have similar treatment methods and principles as VHL syndrome. Treatment principles of renal cancer with VHL syndrome: observe and wait for renal tumor diameter <75px, and consider surgery when the maximum tumor diameter is ≥75px, with NSS as the first choice, including tumor enucleation.