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Introduction The aim of this study was to describe diagnoses and outcomes when women choose to continue a pregnancy despite detection of severe fetal anomaly. Material and methods Retrospective study from a French national registry between 2005 and 2009. Various indication groups were classified based on a scale incorporating mortality and morbidity outcomes. Risk factors for perinatal mortality and predictors of neonatal survival were determined by multinomial logistic regression analyses. Results The overall number of pregnancies that continued after diagnosis of a severe fetal anomaly was 2266 (6.6%). The diagnoses were categorized into life-limiting conditions (34.2%), serious disability anomaly (40.8%), potential intellectual disability (8.6%), other conditions (8.8%), and failure to classify (7.4%). During the study period, there was a significant increase in the number of women who continued pregnancy after their fetus was diagnosed with a life-limiting condition (p = 0.03), and a decrease when the fetus was diagnosed with potential intellectual disability (p = 0.01). Pregnancy outcomes were intrauterine fetal death (17.4%), neonatal mortality (22.3%), neonatal survival (52.7%), or unknown (7.5%). Multinomial logistic regression analysis suggested an increased likelihood of neonatal survival among fetuses diagnosed with serious disability anomaly [59.8%, relative risk (RR) 4.1, 95% CI 3.1-5.7], potential intellectual disability (74.4%, RR 11.5, 95% CI 6.2-21.2), and other conditions (80%, RR 12.6, 95% CI 5.7-27.9) compared with life-limiting conditions (30.1%). Conclusions An increasing proportion of women chose to continue pregnancy after the fetus was diagnosed with a potentially life-limiting condition; survival of children with these diagnoses is not uncommon.