Objective Stickler Syndrome (SS) is associated with eye, joint and orofacial abnormalities. Most cases are dominantly inherited through variants in genes encoding type-II/XI collagen (COL2A1/COL11A1), with patients having up to 78% retinal detachment (RD) risk.1 More recently, rarer cases of recessive SS have been identified, associated with pathogenic variants of genes encoding type-IX collagen (COL9A1-3), but there is limited published data on patients’ phenotype or RD risk. Our study aimed to investigate the RD risk in recessive SS, primarily to determine whether patients would benefit from prophylactic retinopexy. A secondary objective was to explore patient phenotypes, identifying key features which clinicians should identify, leading to genetic testing and early diagnosis. Methods We report 13 cases from 11 families with Type-IX collagen recessive SS, identified from the cohort attending the National Stickler Syndrome Service at Addenbrooke’s Hospital, Cambridge, between 1/1/15-31/12/22. Patients underwent multidisciplinary assessment by ophthalmology, rheumatology and audiology. Results 6/11 families exhibited previously undescribed genetic variants, and 7 had consanguineous parents. Clinical findings included abnormal vitreous architecture and high myopia. 15.4% of patients developed RD secondary to horseshoe retinal tears, with no cases of bilateral RD or Giant Retinal Tears. No patients had cleft palate, 30.8% had midfacial hypoplasia. Hearing loss was more prevalent (91.7%) than that reported for dominant SS. Arthropathy was uncommon but highly variable in manifestation. Conclusions There is currently insufficient evidence to suggest that all patients with recessive Stickler Syndrome require prophylactic retinopexy, and it should only be offered case-by-case according to individual risk assessment.