Despite subtle nuances, all guidelines agree that a family history for VHL and one manifestation or two VHL manifestations in the absence of a family history should raise a clinician's suspicion of VHL disease (Choyke et al., 1995; Hes et al., 2005; Hes & van der Luijt, 2000; Lonser et al., 2003; Maher et al., 2011; Nordstrom‐O'Brien et al., 2010; VHL Alliance, 2010). This evidence concerns the gene VHL and von Hippel-Lindau disease.