Despite the importance of type XI collagen in the homeostasis of joints, rheumatoid arthritis (RA) has not been described in association with variants in COL11A2, and we herein describe a patient with compound heterozygous variants in the COL11A2 gene, who exhibited progressive sensory hearing loss since childhood and diagnosed with RA in her fifties. Here, COL11A2 is linked to rheumatoid arthritis.