Nucleolar and coiled-body phosphoprotein 1 (NOLC1) is a phosphoprotein that transiently associates with the mature nucleolar H/ACA and C/D box small nucleolar ribonucleoproteins (snoRNPs). NOLC1 plays an essential role in the synthesis of rRNA and the biosynthesis of ribosomes. NOLC1 is responsible for the nucleolus construction and rRNA synthesis interacted with TRF2 and mediated the shuttle of TRF2 between the nucleolus and nucleus. Studies suggest that NOLC1 is crucial for normal cell growth, and plays a role in the regulation of tumorigenesis of nasopharyngeal carcinoma (NPC) and demonstrate that both NOLC1 and tumor protein 53 work synergistically to activate the MDM2 promoter in NPC cells. DNA methylation is a key mechanism of silenced NOLC1 expression in human hepatocellular carcinoma cells, and NOLC1 gene hypermethylation of the four CpG dinucleotides is a potential biomarker for hepatocellular carcinoma.