Interstitial lung disease (ILD) is a known complication of Primary Sjögren's syndrome (SS). We report the case of a 56-year-old man with a history of SS (SS-A positive) who was admitted with ILD exacerbation, causing respiratory failure requiring extracorporeal life support. Given the family history of rapidly progressive ILD and mixed connective tissue disease in his brother, the patient was tested for short telomere syndrome (STS) during hospitalization and found to have leukocyte telomere length (LTL) around the first percentile, suggesting the diagnosis of STS. The patient successfully underwent bilateral lung transplantation while being supported by venovenous extracorporeal membrane oxygenation (VV-ECMO). As STS has been associated with the development of ILD, the coexistence of STS and SS in our patient represents a unique scenario. This case also raises awareness of the connection between other connective tissue diseases (CTDs) and STS in patients diagnosed with ILD.