We thank de Boer et al1 for their comments and appreciate their work describing the clinicopathologic spectrum in 78 patients with RVCL-S.2 Their series included 13 patients with a mean age of 35.1 ± 10.6 years without retinopathy, brain lesions, or renal involvement. Their long-term clinical experience elegantly highlights the importance of recognizing RVCL-S as a devastating progressive microvascular disease. In contrast, the 56-year-old proband in our pedigree was brought to the attention of a nephrologist because of advanced kidney involvement without retinal disease or symptomatic neurologic involvement.