Introduction: Data on the outcomes of early-onset twin-twin transfusion syndrome (TTTS), diagnosed before 18 weeks gestational age (GA), are sparse. We aimed to review the diagnosis, management and outcomes of early-onset TTTS. Material and methods: Pregnancy records at a single referral unit 2010-6 were reviewed. In early-onset TTTS cases, data for pregnancy characteristics, interventions and outcomes were collected. PubMed and Scopus databases were searched for studies including pregnant women with early-onset TTTS. The primary outcome measure was livebirths. Results: Case series: 58 cases of early-onset TTTS 2010-6, with full outcome data in 44. Diagnostic criteria were variable. Median GA at intervention was 17 +4 (range 15 +0 -28 +1 ); 67% of patients had laser therapy (39/58). Overall survival: 60% (53/88). At least one livebirth: 86% (38/44), Two livebirths: 34% (15/44); No survivors: 14% (6/44). GA at delivery was 32 +1.5 (range 16 +2 -37 +4 ). Systematic review: 16 studies included (n = 171 pregnancies). Diagnostic criteria varied widely: 79% of studies used Quintero staging. Most offered laser (89%) at median 17 +0 weeks (range 16 +0 -21 +6 ). GA at delivery was 23 +0 -39 +5 weeks. Overall survival: 69% (129/186). At least one livebirth: 74% (127/171). Two livebirths: 59% (55/93). No survivors: 26% (44/171). Conclusions: In comparison with the commonly accepted overall survival for TTTS treated after 18 weeks of 60-90%, outcomes in early-onset TTTS were at the lower bound of this range. Gestational age at intervention is similar to that of later onset TTTS, indicating a lack of therapeutic options when a diagnosis is made before 18 weeks.