Purpose To evaluate if neonates delivered after 34(0/7) weeks from mothers diagnosed with gestational diabetes (GD) are exposed to an increased risk of neonatal severe respiratory distress syndrome (SRDS). Methods Women with singleton pregnancy in labour after 34(0/7) weeks of gestation or admitted for planned caesarean section and who had been systematically screened for GD were eligible to participate to this prospective cohort study. Diagnosis of SRDS was defined by the association of clinical signs of early neonatal respiratory distress, with consistent radiologic features and requiring mechanical ventilation with a fraction of inspired oxygen (FiO(2)) > 0.25 for a minimum of 24 h and admission to neonatal intensive care unit. Results A total of 444 women were included. GD was diagnosed in 60 patients (13.5%). A neonatal SRDS was diagnosed in 32 cases (7.2%). Compared to others, neonatal SRDS was significantly more often observed in neonates from women diagnosed with GD: 12 (20%) vs. 20 (5.2%), respectively (p < 0.001). Women whose neonates presented neonatal SRDS were significantly more likely to be obese (p = 0.002), to have undergone a caesarean section (p < 0.001) and to have received corticosteroids therapy before 34(0/7) weeks (p = 0.013). In multivariate analysis, GD was identified as an independent risk factor of neonatal SRDS (aOR 3.6; 95% CI 1.5-8.6; p = 0.005). Other risk factors were maternal obesity (aOR 2.8; 95% CI 1.1-7.1; p = 0.029) and assisted vaginal delivery (aOR 5.5; 95% CI 1.9-15.9; p = 0.002). Conclusions GD is an independent risk factor of neonatal SRDS after 34(0/7) weeks.