The patient characteristics and outcomes associated with autosomal dominant polycystic kidney disease (ADPKD) have not been characterized in elderly population. The purpose of theis study was to delineate clinical presentation and outcome of ADPKD in elderly patients. We performed a retrospective study. Thirty four elderly patients were diagnosed with ADPKD between 1975 and 2005. The diagnosis of ADPKD was made using family history and ultrasound. There were 21 (61.7%) males and 13 (38.3%) females. The mean age at the time of diagnosis of ADPKD was 69.74 ± 3.36 years (range, 65-79 years). The earliest clinical features were renal failure in 67.6%, back pain in 38.2%, and hypertension in 17.6%. Most common form of presentation was hypertension in 70.6%. Kidneys were palpable in 26.5%, and liver was palpable in 32.4%. Left ventricular hypertrophy confirmed by transthoracic echocardiography was found in 5(1.5%) patients. Three (0.8%) patients had colonic diverticula and two (0.6%) had neurological manifestation. Twenty six 26 patients (76.5%) patients had end stage renal disease and 30 patients (88.23 %) patients had anemia. Median follow-up period for all patients was 17.9 months (range, 1-120 months). Two (0.6%) patients developed renal carcinoma while five patients (14.7%) died within six years following diagnosis Anemia was the strongest and an independent predictor of poor renal outcome. (p<0.03). The diagnosis of ADPKD in elderly patients was made late in most cases, with patients already at end stage renal disease. Anemia is risk factor of poor renal prognosis. Eearly diagnosis and efforts at prevention of the disease progression and complications are most essential.