- Introduction: Deviation in the development of the female reproductive organs from the normal anatomy has been shown to have an impact on obstetrical outcomes and neonatal morbidity. Material and methods: In this retrospective population-based cohort study, short-term neonatal morbidity and mortality were compared in pregnancies of women with and without uterine anomalies. The analysis included deliveries that occurred between the years 1991 and 2013 in a tertiary medical center. Statistical analysis included multiple logistic regression models. Results: During the study period, 256 299 deliveries met the inclusion criteria; 0.49% (n = 1251) of which occurred in women diagnosed with Müllerian anomalies. In the regression model, Müllerian anomalies were noted as an independent risk factor for placental abruption (adjusted odds ratio, 1.9; 95% confidence interval, 1.3-2.8; p = 0.001), intrauterine growth restriction (adjusted odds ratio, 1.9; 95% confidence interval, 1.5-2.4; p < 0.001), pathological presentation (adjusted odds ratio, 13.5; 95% confidence interval, 11.9-15.1; p < 0.001) and cesarean delivery (adjusted odds ratio, 13.4; 95% confidence interval, 11.5-15.6; p < 0.001) while controlling for multiple confounders. Perinatal mortality, however, was not found to be increased in the exposed group in a model controlled for gestational age and weight (adjusted odds ratio, 0.6; 95% confidence interval, 0.4-1.02; p = 0.061). Conclusion: Women with Müllerian anomalies are at an increased risk for multiple adverse pregnancy outcomes, including preterm delivery and intrauterine growth restriction. Perinatal mortality, however, is not increased when controlled for gestational age and weight suggesting that mortality in these pregnancies is mediated by preterm delivery and small for gestational age.