The term “fatty transformation of the kidney” first appeared in the literature in 1883. As the global epidemic of overweight and obesity continues to escalate, the biopsy incidence of obesity-related glomerulopathy has risen correspondingly, establishing fatty kidney disease as an increasingly recognized clinical entity within chronic kidney disease. Our understanding of how increased fat accumulation in and around the kidney contributes to the complex pathogenesis of fatty kidney disease has substantially improved. In this review article, we summarize the current literature on fatty kidney disease. We also present recent advances in treatment strategies such as glucagon-like peptide-1 receptor agonists and highlight the importance of a multidisciplinary team approach for the early detection and effective management of patients with chronic kidney disease with overweight and obesity.