We compared patients with and without cholelithiasis and found that the frequency of UGT1A1 alleles (TA)6 was lower, and that of alleles (TA)7 was significantly higher in patients with cholelithiasis (p = 0.002, OR = 2.74, 95% CI 1.42–5.25). These data suggest a possible relationship between the UGT1A1 locus and the occurrence of cholelithiasis in patients with SCA and bTH and most probably other hemoglobinopathies. The gene discussed is UGT1A1; the disease is autosomal dominant cerebellar ataxia.