Hereditary papillary renal cell carcinoma (HPRCC) is an autosomal dominant syndrome characterized by the occurrence of bilateral and multifocal, classic type papillary renal cell carcinomas. First described in 1994, these tumors presented with a tubulopapillary histomorphology with distinct cytogenetic features.1, 2 HPRCC is an extremely rare familial disorder with an unknown prevalence. Compared to other hereditary renal cell carcinomas, HPRCC tend to be of indolent nature and garner a less aggressive surgical follow up.3 Much work has been done in the recent decades elegantly elucidating the molecular basis of its tumorigenesis and it has been found that patients with HPRCC harbor missense germline mutations of proto-oncogene MET.4

Herein, we will provide a review of this syndrome and its underlying genetic and molecular features, clinical manifestations, histomorphological characteristics and finally, compared to its sporadic counterpart tumors, lessons learned from studying the syndrome.