Substantiating concern about an increased risk of malignancy in a subset of patients with SMARCB1-positive schwannomatosis, Hulsebos et al. (2016) reported a type 1 papillary renal cell carcinoma (pRCC1) in a schwannomatosis patient with a germline duplication of SMARCB1 exon 7 (c.796-2246_9861 +5250dup7686) resulting in a premature stop codon (p.Glu330*). The gene discussed is SMARCB1; the disease is schwannomatosis.