Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant genetic disorder characterized by der-matofibroma, renal tumor, pulmonary cyst and spontaneous pneumothorax. At present, diagnosis of BHD syn-drome is based on clinical manifestations, imaging features and genetic evidence. Mutations in Folliculin (FLCN), a tumor suppressor gene, are the main genetic lesion that causes BDH syndrome. The FLCN protein can form a complex with FLCN-interacting protein 1 (FNIP1), FNIP2 and adenosine monophosphate-activated protein kinase (AMPK), acting on occurrence and development of BDH syndrome by regulating mammalian target of rapamycin (mTOR) pathway. Recent studies have found that obvious differences exist in BHD syn-drome-associated renal tumors, sporadic renal tumors and other genetically related renal tumors. Herein, the molecular mechanism and pathological characteristics of BHD syndrome-associated renal tumors were sum-marized, and the differences between BHD syndrome-associated renal tumors and other renal tumors were discussed, hoping to give some reference in clinical diagnosis of BHD syndrome-associated renal tumors and development of effective targeted therapies in addition to surgery.