Antisynthetase syndrome (anti-SS) is a rare systemic autoimmune disease characterised by autoantibodies against aminoacyl-tRNA synthetases manifesting as one or more components of the classic triad: interstitial lung disease, arthritis and myositis. While it is well-recognised that autoimmune rheumatological disorders in general can contribute to multiple pregnancy complications, very little is known about how anti-SS itself affects pregnancy outcomes. Described here is the case of a 26-year-old pregnant woman with anti-SS whose pregnancy course was complicated by placental dysfunction and subsequent extremely premature delivery at 24 weeks' gestation. This report presents a review of the literature to date and discusses potential pregnancy complications associated with anti-SS and their subsequent targeted management.