Background Vascular Ehlers‐Danlos syndrome (vEDS) is a hereditary connective tissue disorder associated with an elevated risk of vascular, uterine and digestive complications. Managing pregnancy in this context can be a challenge. Objectives To systematically review the literature data on the complications in pregnancy associated with vEDS. Search strategy We searched the Pubmed Medline and Embase databases for articles using the following terms “vascular Ehlers‐Danlos syndrome” or “vEDS” AND “pregnancy”. Selection criteria Women with vEDS. Data collection and analysis We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS. Main results A total of 121 publications were screened, with six (accounting for 412 pregnancies) included in our review. Of the women included in this sample, 30% were infertile. The miscarriage rate was 13.8% (57/412) and 8.8% of the live births were premature. Obstetric anal sphincter injuries occurred in 11.3% (23/203) of the deliveries. The maternal mortality rate per pregnancy was 5.7%. Conclusions Women with vEDS present an elevated risk of uterine rupture, vascular events, digestive events and death during pregnancy. Women appear to be most at risk during the peripartum period; to avoid expulsive efforts, a caesarean section should be scheduled at 37 weeks of gestation.