Nephrectomy is one of the commonest surgeries done in urology for kidney lesions. Routinely, kidney specimens from autopsy are being sent to look for morphological changes occurring in both natural and unnatural deaths. Primitive neuroectodermal tumor/Ewing’s Sarcoma (PNET/ES), a member of Ewing’s sarcoma family is an aggressive tumor rarely observed in kidney principally occurs in young patients and is very rare in older patients. Hence this study was undertaken to contemplate the histomorphological pattern of kidney lesions in nephrectomy and autopsy specimens with an accentuation on Primitive Neuroectodermal tumor in Kidney which is a rare disease.This study was carried out in the Dept of Pathology, MMCRI, Mysore, Karnataka. All the nephrectomy and autopsy specimens received over a period of one year (June 2018 to May 2019) were included. A total of 38 cases were studied during this period. Out of 38 cases, 29 were non-neoplastic and 9 were neoplastic lesions. Among non-neoplastic, 19 were chronic pyelonephritis and in neoplastic, renal cell carcinoma clear cell type (5 cases) was the commonest with a case of renal PNET (rPNET).The present study provides a fair insight into the histological patterns of lesions in nephrectomy and autopsy specimen as they are liable for a great deal of morbidity and mortality. Histomorphological study of kidney lesions should be done thoroughly for early identification and prevention of progression of disease, and for prognosis and treatment.Also features infrequent occurrence of PNET/ES of the kidney which is uncommon and is significantly rarer in older patients.