Background: Nephrectomy is a common urological procedure in urological practice. It is indicated in patients presenting with a wide range of clinical conditions ranging from symptomatic chronic infections, obstruction, calculus disease, and severe traumatic injury and neoplastic conditions. In India management of chronic kidney diseases and neoplasms are posing a great challenge as dialysis and kidney transplantations are expensive for the patients. Aims and Objectives: 1) To study the histopathological features of lesions in nephrectomy specimens 2) To evaluate the frequency of different pathological lesions in nephrectomy specimens 3) To correlate the histopathological diagnosis with the clinical features. Materials and Method: This was a prospective study conducted in the conducted in the department of pathology, M.S. Ramaiah Medical College and Hospitals, Bangalore. The study included all nephrectomy specimens received for routine histopathological examination over a period of 2 years (from July 2014 to June 2016). All the nephrectomy specimens were sent to histopathology section in 10% formalin. All specimens were subjected to detailed gross examination and representative tissue bits were taken. For microscopic examination, 4-5 µm thick paraffin sections were cut and stained with haematoxylin and eosin (H &E). Relevant patient particulars including age, sex, affected side, clinical presentation and investigations such as Computed Tomography, Ultrasonography etc were retrieved from the case files. Results: Out of 62 patients, 38 cases were males and 24 cases were females with male: female ratio of 1.58:1. The mean age was 43.93±18.67 years with the youngest patient aged 1 year and the oldest patient aged 75 years. The mean age of the males was 42.92±19.50 years and the mean age of females was 45.54±17.56 years. Neoplastic lesions accounted for 51.63% of the cases and were more common than non-neoplastic lesions of the kidney which accounted for 48.38% of the cases. Renal cell carcinoma was the commonest lesion of the kidney, accounting for 56.25% of the neoplastic lesions and 29.03% of all renal lesions followed by Wilms’ tumor (6.45%, 4/62). Other neoplastic lesion encountered were Transitional cell carcinoma (4.84%, 3/62), Renal oncocytoma (4.84%, 3/62) Angiomyolipoma (4.84%, 3/62) and Squamous cell carcinoma (1.61%, 1/62). Chronic pyelonephritis was the commonest non-neoplastic lesion encountered in the present study which accounted for 43.33% of all non neoplastic lesions and 20.97% of all the lesions of the kidney followed by Hydronephrosis (16.13%, 10/62). Other non neoplastic lesions Tuberculous pyelonephritis (4.84%, 3/62), Xanthogranulomatous pyelonephritis (1.61%, 1/62), Cystic renal dysplasia (1.61%, 1/62), End stage kidney disease (1.61%, 1/62), Renal hydatid cyst (1.61%, 1/62). Conclusion: The present study provides a fair insight into the histopathological patterns of lesions in nephrectomy. Neoplastic lesions were more common than nonneoplastic lesions of the kidney. Renal cell carcinoma was the commonest neoplastic lesion and chronic pyelonephritis was the commonest nonneoplastic lesion in this series. The histopathological diagnosis generally correlates well with the pre operative clinical diagnosis, however some benign lesion may be misdiagnosed clinically as malignant and some malignant lesions may be misdiagnosed clinically as benign. As renal lesions may be misdiagnosed clinically, it is mandatory that every nephrectomy specimen be subjected to a detail histopathological examination to ensure proper post-operative management.