While patients who were homozygous for the c.254-649T>G variant were reported to have a severe USH type1 phenotype with early-onset hearing loss, the index case we are reporting here, who has a compound heterozygous for p.(N48K) (known to cause USH3 in homozygous and compound heterozygous states) and c.254-643G>T, has non-syndromic RP at the age of 51 years. The gene discussed is CLRN1; the disease is hearing loss disorder.