Current understanding of clinical practice and care for maternal kidney disease in pregnancy in Australia is hampered by limitations in available renal-specific datasets. To capture the epidemiology, management, and outcomes of women with significant kidney disease in pregnancy and demonstrate feasibility of a national cohort study approach. An Australian prospective study (2017-2018) using a new kidney disease-specific survey within the Australasian Maternity Outcomes Surveillance System (AMOSS). Women who gave birth with acute kidney injury (AKI), advanced chronic kidney disease (CKD), dialysis dependence or a kidney transplant were included. Demographic data, renal and obstetric management, and perinatal outcomes were collected. Among 58 case notifications from 12 hospitals in five states, we included 23 cases with kidney transplant (n = 12), pre-existing CKD (n = 8), newly diagnosed CKD (n = 2) and dialysis (n = 1). No cases of AKI were reported. Reporting rates were better in states with study investigators and, overall, cases were likely under-reported. Nearly 35% of women had a non-delivery-related antenatal admission. Nephrology involvement was 78.3% during pregnancy and 91% post-partum. Adverse events were increased, including pre-eclampsia (21.7%), and preterm birth (60.9%). Women had high rates of aspirin (82.6%) and antihypertensive (73.9%) use, indwelling catheter for labour/delivery (65.2%), caesarean delivery (60.9%), and blood transfusion (21.7%). This first-ever Australian prospective study of significant kidney diseases in pregnancy provided novel insights into renal-specific clinical patterns and practices. However, under-reporting was likely. Future studies need to overcome the challenges of case identification and data collection burden.