Von Hippel-Lindau syndrome with unusual presentations in two brothers

Two cases are reported of von Hippel-Lindau syndrome in two brothers. One had an asymptomatic adrenal pheochromocytoma (probably bilateral) and a synchronous metastatic hypernephroma which presented as a rather "innocent" renal colic. The second asymptomatic brother underwent urologic investigation which revealed multifocal tumors of the kidney. High clinical suspicion based on family history only is mandatory. The literature related to von Hippel-Lindau syndrome is reviewed.