Maruo et al[20] reported a triple homozygous mutation in UGT1A1 gene [T-3279G, A(TA)7TAA, and H39D] in a CNS-II patient, and co-occurrence of 3 other mutations in a family with CNS-I and GS.[21] Our report and other studies[20–22] further emphasized the importance of family screening among UCH cases with triple/more alleles. This evidence concerns the gene UGT1A1 and Gerstmann syndrome.