OBJECTIVES: There is limited real-world information on resource utilisation, costs and productivity in patients with ADPKD. The objective of this study was to estimate the annual direct and indirect costs of patients with ADPKD by severity of the disease [chronic kidney disease (CKD) stages 1–3, 4–5, dialysis, and transplant recipients], and describe the resource utilisation during the 12-month period around dialysis initiation and transplant procedure, respectively. METHODS: A cross-sectional study of ADPKD patients was undertaken April–December 2014 in Denmark, Finland, Norway and Sweden. Data on medical resource utilisation were extracted from medical charts and patients were asked to complete a self-administered survey, which included the Work Productivity and Activity Impairment Questionnaire. RESULTS: A total of 266 patients were contacted, 243 (91%) of whom provided consent to participate in the study. Average total annual costs amounted to €9,919 in CKD 1–3, €16,761 in CKD 4–5, €74,015 in dialysis patients and €31,496 in transplant recipients. Indirect costs were substantial in earlier stages of the disease, accounting for 72% and 73% of total costs in the CKD 1–3 and CKD 4–5 strata, respectively. Direct medical costs amounted to €60,514 in dialysis patients and €17,773 in transplant recipients. Among those who were employed, the overall work productivity loss ranged between 9% in CKD stage 1–3 to 42% in dialysis patients. An average of 16.5 hospital days and 10.4 outpatient visits were observed in the 12-month period around dialysis initiation. Around the transplant procedure, the average number of hospital days and outpatient visits were 29.9 and 16.9, respectively. CONCLUSIONS: ADPKD is a progressive disease associated with increasing costs as the patient progresses to CKD stage 4–5, dialysis and transplant. Substantial savings and productivity gains could be made by delaying the progression of ADPKD to end stage renal disease.