Aim: The aim of this study to identify the different histopathological pattern of neoplastic lesions in nephrectomy specimens. Materials and Methods: This was a retrospective record analysis done over a period of three years (January 2021 to December 2023). All nephrectomy specimens with a diagnosis of renal tumor received in the Department of Histopathology, National Institute of Kidney Diseases and Urology, Dhaka were recorded from histopathology registers and analyzed for pattern and grading of renal tumors. Results: A total of 230 renal tumors were studied. Of the 230 renal tumors encountered during the study period, 205 cases (89.13%) were diagnosed as malignant tumors and 25 cases (10.87%) as benign tumors. There were 151 male and 79 were female cases (male-Female ratio 1.91:1) and Mean age 62 years. Renal tumors occurred most commonly in 6th and 7th decades and least in 2nd decade. In our study, mean age of patients with benign tumors was 52 years and that for patients with malignant tumors was 64 years. Renal cell carcinoma was the commonest tumor 170 (73.91%) followed by Wilm’s tumor 18 (7.83%) followed by transitional cell carcinoma 06 (2.61%). Neuroblastoma and sarcomatoid renal cell carcinoma showed in 5 (2.17%) and 4 (1.74%) cases respectively. The most frequent subtype of RCC were clear cell carcinoma 135 (79.41%) followed by papillary renal cell carcinoma 17 (10%). Relatively serious collecting duct carcinoma and most serious renal medullary carcinoma were found in 06 (3.53%) and 04 (2.35%) cases respectively. A total of 152 cases of clear cell renal cell carcinoma and papillary renal cell carcinoma which were graded 1–3 based on nucleolar prominence, whereas grade 4 was defined by the presence of tumor cells with sarcomatoid and/or rhabdoid morphology and/or tumors containing tumor giant cells or showing extreme nuclear pleomorphism. Most frequent grade in this study group was grade-2 (53.29%) followed by grade-3 (33.35%) where the least frequent grade was grade-4 which is most dangerous. Conclusions: This retrospective study showed distribution and grading of renal tumors in the nephrectomy specimens performed at our institution. The results obtained from our study were comparable with available data from other countries.