Treatment with ursodeoxycholic acid, glucocorticoids, and mesalazine led to adequate control of intestinal symptoms but only partial improvement in liver biochemistry.<h4>Conclusion</h4>This case reports a rare instance of primary sclerosing cholangitis complicated with ulcerative colitis in a young female, who was detected to have heterozygous mutations in the UGT1A1 and SLC25A13 genes. This evidence concerns the gene UGT1A1 and ulcerative colitis.