INTRODUCTION: We assessed the risk of adverse pregnancy and birth outcomes and birth defects among women living with HIV (WLHIV) on antiretroviral therapy (ART) and HIV-negative women. METHODS: We analyzed data on live births, stillbirths, and spontaneous abortions during 2015-2021 from a hospital-based birth defects surveillance system in Kampala, Uganda. ART regimens were recorded from hospital records and maternal self-reports. Using a log-binomial regression model, we compared the prevalence of 16 major external birth defects and other adverse birth outcomes among WLHIV on ART and HIV-negative women. RESULTS: A total of 203,092 births were included from 196,373 women of whom 15,020 (7.6%) were WLHIV on ART. During pregnancy, 15,566 infants were primarily exposed to non-nucleoside reverse transcriptase inhibitor-based ART (n = 13,614
87.5%). After adjusting for maternal age, parity, and number of antenatal care visits, WLHIV on non-nucleoside reverse transcriptase inhibitor were more likely than HIV-negative women to deliver preterm (adjusted prevalence ratio [aPR] = 1.27, 95% confidence interval: 1.21 to 1.32), post-term (aPR = 1.23, 95% CI: 1.16 to 1.32), or small for gestational age infants (aPR = 1.35, 95% CI: 1.30 to 1.40). Spina bifida was more prevalent among infants born to WLHIV on ART periconceptionally compared with HIV-negative women (aPR = 2.45, 95% CI: 1.27 to 4.33). The prevalence of the other selected birth defects was similar between infants from WLHIV on ART and HIV-negative women. CONCLUSIONS: In Uganda, WLHIV on ART were more likely than HIV-negative women to experience selected adverse birth outcomes. Further surveillance of maternal ART exposure, including by drug class and ART regimen, is needed to monitor and prevent adverse birth outcomes in WLHIV.