The purpose of this investigation was to study the risk of intrauterine growth restriction in human immunodeficiency virus (HIV)-infected women and to describe the associated risk factors. A cohort study was performed among HIV-infected women who delivered in a single tertiary centre in Barcelona, Spain, from January 2006 to December 2011. Consecutive singleton pregnancies delivered beyond 22 weeks of pregnancy were included. Intrauterine growth restriction (IUGR) was defined as a birth weight below the 10th customised centile for gestational age and IUGR babies were compared to non-IUGR newborns. Intrauterine Doppler findings were described among IUGR foetuses. Baseline characteristics, HIV infection data and perinatal outcome were compared between groups. The results were adjusted for potential confounders. A total of 156 singleton pregnancies were included. IUGR occurred in 23.4 % of cases (38/156). In two-thirds of the cases detected before birth, Doppler abnormalities compatible with placental insufficiency were observed. IUGR pregnancies presented a worse perinatal outcome, mainly due to a higher risk of iatrogenic preterm delivery [adjusted odds ratio 6.9, 95 % confidence interval (CI) 1.4-33.5]. IUGR foetuses also had a higher risk of emergent Caesarean section and neonatal intensive care unit admission. No cases of intrauterine foetal death occurred. A high rate of IUGR was observed among HIV pregnancies, and it was associated with adverse perinatal outcomes, mainly iatrogenic preterm and very preterm birth due to placental insufficiency. Our results support that ultrasound detection and follow-up of IUGR foetuses should be part of routine antenatal care in this high-risk population to improve antenatal management.