The relationship between familial Mediterranean fever (FMF) and pregnancy outcomes remains unclear. This systematic review and meta-analysis aimed to clarify this association.Electronic databases-the PubMed, Web of Science, Cochrane, and Embase-were searched on 20 December 2022, using specific search terms. Case-control, cohort, and randomised clinical trial studies comparing patients with FMF and healthy controls were considered eligible. We excluded systematic reviews, meta-analyses, case series with fewer than five cases, republished articles without new findings on pregnancy outcomes, studies targeting paternal FMF, and those not published in English. Odds ratios (OR) and 95% confidence intervals (CI) summarised the results using a random-effects model. This study was registered in the University hospital Medical Information Network Clinical Trials Registry (Japan) as UMIN000049827.The initial electronic search identified 611 records, of which 9 were included in this meta-analysis (177 735 pregnancies, 1,242 with FMF, and 176 493 healthy controls). FMF was significantly associated with increased odds of preterm deliveries (OR, 1.67; 95% CI, 1.05-2.67; I2 = 22%) and insignificantly associated with increased odds of fetal growth restriction (OR, 1.45; 95% CI, 0.90-2.34; I2 = 0%) and hypertensive disorders during pregnancy (OR, 1.28; 95% CI, 0.87-1.87; I2 = 0%).FMF was significantly associated with preterm delivery and insignificantly associated with fetal growth restriction and hypertensive disorders. All of the included studies were observational studies. Treatment characteristics were not fully collected from the articles, and further analysis of treatments for FMF in pregnancy is still warranted.