Polycystic kidney disease (PKD) has an autosomal dominant mode of inheritance. Despite this 25% of patients will have no known family history and these cases are thought to be due to de novo mutations in the causative genes. PKD can present asymptomatically and should be considered in any patient with renal cysts noted on abdominal imaging, especially if there is a history of hypertension. Patients with PKD may present with hypertension before a decline in eGFR is noted. Cyst haemorrhage, rupture or infection can all present with acute loin pain. PKD can also cause a number of extrarenal complications.