Autosomal Dominant Polycystic Kidney Disease (ADPKD) presents a significant burden globally, including in Tunisia. This retrospective study aimed to characterize ADPKD cases in Tunisia, focusing on clinical manifestations, therapeutic interventions, and prognostic markers influencing progression to End-Stage Renal Disease (ESRD). Data from 107 patients over 21 years revealed a mean age of 53.43 years and a predominance of renal failure as the primary clinical presentation (47%). Notably, a substantial proportion (42/59) of patients without advanced renal failure at diagnosis progressed to ESRD during follow-up, with identified prognostic factors including anemia, urinary tract infections, kidney size, and cyst volume. These findings underscore the importance of early detection and personalized management strategies in ADPKD.