Point mutations in POT1, particularly those affecting its oligonucleotide/oligosaccharide-binding (OB) fold domain (POT1 c.1432G > T (p.Cys476Phe) and c.1166C > T (p.Ala389Val), have been found in malignancies including chronic lymphocytic leukaemia (CLL), melanoma, and gliomas. Here, POT1 is linked to central nervous system cancer.