The Present Study was conducted in department of Medicine, Govt. Medical College, jammu, Where a total of 41 patients—29 males and 12 females—fulfilled the inclusion criteria of ADPKD, were gathered during the period of 1 year starting from Nov. 2011 to Oct. 2012. All the patients were subjected to a detailed history, clinical examination and laboratory investigations. X-ray chest (PA view), ECG and ultrasound of abdomen for kidneys, liver and spleen were done. Intravenous pyelogram and CT scan of abdomen was done when a definitive diagnosis of (ADPKD) could not be made on abdominal ultrasound. Echocardiography was done to evaluate cardiac murmurs and associated mitral valve prolapse, based on standard criteria. Male to female patients with ADPKD was 2.42:1. Maximum 17 (41.5%) patients of both gender were seen in 30 - 40 years age group, Family history of ADPKD was present in 18 (43.9%) patients; Hypertension, alone or in combination with renal failure, was present in 65.8% patients; Hypertension alone was present in 19 (46.3%) patients;8 (19.5%) patients with hypertension had renal failure; Low back pain was present in 24 (58.5%) and abdominal pain in 22 (53.7%) patients; 15 (36.6%) patients presented with at least one episode of gross haematuria; Headache was experienced by 18 (43.9%) patients. On clinical examination, 24 (58.5%) were found to have palpable kidney and 10 (24.4%) had palpable liver. Spleen was palpable in 1 (2.4%) patient, Murmur of mitral valve prolapse was found in 2 (4.9%) Patients; 3 (7.3%) patients having left ventricular hypertrophy; mean Hb was 11.2 g/dL. The liver cysts were found in 24.4% of the patients; Out of 10 (24.4%) patients with hepatic cyst involvement, 1 patient each was found to have evidence of portal hypertension and evidence of hepatic cyst infection. In the present study, hypertension was most common presentation of this disease. So, control of hypertension is very important to prevent progression of this disease. Patients who are detected to have ADPKD should be regularly followed-up to prevent further progression by timely intervention. Also, family members of patients should be screened for disease and initiate treatment as early as possible.