This chapter focuses on the pathology of the kidney, detailing a range of congenital, acquired, and neoplastic conditions. Structural anomalies such as horseshoe kidney, multicystic dysplastic kidney, and autosomal recessive polycystic kidney disease (ARPKD) are explored. Glomerular disorders, including diabetic glomerulosclerosis, dense deposit disease (DDD), and Kimmelstiel–Wilson disease, are analyzed for their clinical and histological implications. Vascular complications such as renal infarction, polyarteritis nodosa (PAN), and thrombotic conditions such as hemolytic–uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are examined. The chapter also discusses tubulointerstitial diseases, including acute tubular necrosis (ATN), urate nephropathy, and xanthogranulomatous pyelonephritis, alongside chronic kidney disorders such as nephrosclerosis and end-stage renal disease (ESRD). Renal transplant pathology, including acute transplant rejection and Banff classifications, is highlighted. Neoplasms like renal cell carcinoma and Wilms tumor are explored, with emphasis on genetic syndromes such as WAGR syndrome and Denys–Drash syndrome. Through comprehensive insights into kidney pathology, this chapter provides a solid foundation for understanding renal diseases.