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OBJECTIVE: The primary objective of this systematic review was to evaluate pregnancy outcomes following at least 1 episode of diabetic ketoacidosis in patients with gestational or pregestational diabetes mellitus. DATA SOURCES: An electronic search of English-language publications in PubMed, Embase, Web of Science, and Google Scholar covered the period from January 1990 to June 2023. STUDY ELIGIBILITY CRITERIA: The eligibility criteria included randomized clinical trials or observational studies (ie, cohort studies, case-control studies) of patients with diabetic ketoacidosis in pregnancy and evaluation of maternal or neonatal outcomes. Systematic reviews, expert reviews, case reports, case series, animal studies, non-peer-reviewed publications, abstract-only publications, and publications without available full-text articles were excluded. METHODS: Each study was reviewed in detail by 2 coauthors. The quality of each study was appraised using the Cochrane RoB 2 risk-of-bias tool for randomized trials and the JBI Critical Appraisal tools for cohort and case-control analyses. RESULTS: Of the 1313 studies identified through data search, 7 met the final inclusion criteria. Study heterogeneity precluded any meta-analysis. High rates of adverse maternal and neonatal outcomes were found among those with at least 1 episode of diabetic ketoacidosis in pregnancy, with the following incidence rates: 7% to 35% for stillbirth, 49% to 64% for cesarean delivery, 25% to 83% for preterm birth, and 40% to 65% for neonatal intensive care unit admission. The most recent studies note a stillbirth rate of 7% to 31%, with most analyses finding a rate <17%. CONCLUSION: There is a paucity of well-designed studies evaluating pregnancy outcomes associated with diabetic ketoacidosis. Maternal and fetal outcomes following diabetic ketoacidosis remain poor, although stillbirth may be incrementally decreasing over time. As diabetes care and diabetic ketoacidosis management advance, future retrospective and prospective analyses should evaluate the management and outcomes of diabetic ketoacidosis. El resumen esté disponible en Español al final del artículo.