Purpose <p>To systematically review the anatomical and functional outcomes of vitreoretinal surgery for familial exudative vitreoretinopathy (FEVR)-associated retinal detachment (RD).</p> Methods <p>A systematic review and meta-analysis were conducted following a PROSPERO-registered protocol. The Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, EMBASE, Ovid, and Medline databases were searched for cohort studies and case series evaluating scleral buckling (SB), pars plana vitrectomy (PPV), or combined procedures for FEVR. Primary outcomes were retinal reattachment rates and postoperative best-corrected visual acuity (BCVA). Secondary outcomes included complication and reoperation rates.</p> Results <p>Nineteen studies involving a total of 682 eyes were included. The overall pooled primary retinal reattachment rate was 0.77 (95% Confidence Interval (CI): 0.71–0.83; I² = 59.8%). Subgroup analysis demonstrated a pooled primary reattachment rate for SB of 0.90 (95% CI: 0.70–1.00; I² = 12.7%) and for PPV of 0.71 (95% CI: 0.60–0.80; I² = 49.1%). The pooled reoperation rate was 0.24 (95% CI: 0.08–0.45). Qualitative analysis showed that while BCVA outcomes were heterogeneously reported, they generally improved postoperatively. Surgical success was highly dependent on disease stage.</p> Conclusion <p>Surgical management of FEVR-associated RD achieves high primary reattachment rates. While both SB and PPV yield favourable outcomes, the choice of procedure should be tailored to disease severity and patient characteristics. The evidence suggests SB may be preferred for uncomplicated rhegmatogenous detachments, while PPV is often necessary for more advanced and complex cases.</p>