Autosomal dominant polycystic kidney disease (ADPKD) affects up to 1 in 1000 people. The disease is characterized by the progressive development of cysts throughout the renal parenchyma due to inherited pathogenic variants in genes including or and eventually leads to gradual loss of renal function, along with manifestations in other organ systems such as hepatic cysts and intracranial aneurysms. ADPKD management has advanced considerably in recent years due to genetic testing availability, pre-implantation genetic diagnosis technology and new therapeutic agents. Renal disease in pregnancy is recognised as an important risk factor for adverse maternal and fetal outcome. Women with ADPKD and health professionals face multiple challenges in optimising outcomes during the pre-pregnancy, pregnancy and post-partum periods.