Introduction and aim: Patients with haemophilia (PWH) could have higher prevalence of urolithiasis according to some studies compared with age-matched males. There is a lack of data regarding risk factors for urolithiasis in adult PWH patients. The aim of this work is to investigate the prevalence of urolithiasis in adult PWH and associated risk factors. ----- Methods: We cross-sectionally evaluated 92 adult PWH, from one centre, UHC Zagreb. Presence of urolithiasis was assessed by ultrasound, while all patients had a medical history and samples were taken for blood and urine laboratory tests.----- Results: Prevalence of urolithiasis was significantly higher among PWH than reported prevalence in Croatian population (10,9 % vs. 5,9 %; P = 0,042). Similarly, occurrence of urolithiasis during course of disease was significantly higher than estimated lifetime cumulative incidence of urolithiasis in Croatian population (25 % vs 12 %; P = 0,001). Neither current presence of urolithiasis, nor urolithiasis previously recorded during course of the disease showed statistically significant associations with type of hemophilia, severity of hemophilia, use of plasma derived factors or recombinant factors (P > 0,05 for all associations). In multivariate analyses, arterial hypertension, presence of inhibitors, hypercalciuria and serum bilirubin were identified as independent predictors of current urolithiasis (P < 0,05), whereas haematuria (P = 0,051) and prior urinary infections (P = 0,059) were also recognized as relevant factors. Similarly, arterial hypertension, presence of inhibitors and history of macrohematuria were recognized as independent predictors of history of urolithiasis (P < 0,05). ----- Conclusion: Adult PWH have more frequent urolithiasis which can be considered as haemophilia-related disease. Risk factors indicate non-immunological and immunological kidney damage and hypercalciuria as the main etiological mechanisms of urolithiasis in these patients.