Only two families, one large family with multiple cases of CRC and a truncating RPS20 variant (c.147dupA, p.Val50SerfsTer23), and one small family with a splice site variant shown to disturb normal splicing (c.177+1G>A), have been reported in addition to two individuals with early onset CRC and without published family cancer history (Nieminen et al., 2014; Broderick et al., 2017; Thompson et al., 2020). The gene discussed is RPS20; the disease is cancer.