Background: Diabetic kidney disease (DKD) is regarded as the leading reason for inducing end-stage renal disease (ESRD), representing (34.7%) of hemodialysis patients in Egypt. Also, the prevalence of non diabetic kidney disease (NDKD) is high in type 2 diabetes mellitus (T2DM). Consequently, renal biopsy is considered the gold standard for determining NDKD in T2DM. This study investigated the potential reasons for triggering kidney disease in T2DM cases with atypical presentations of DKD. Patients and Methods: The present study was conducted in the Department of Internal Medicine, Nephrology Unit, Assiut University Hospital, Egypt, during the interval from January 2016 to May 2020. We recruited 60 patients with T2DM and investigated for the atypical presentation of DKD in this study. Subjects underwent laboratory investigations, examination, detailed history, special investigations when indicated, fundus examination, and renal biopsy. Results: The current study showed that the NDKD either alone or coexisting with DKD, representing 81.7% of the studied patients. Subjects were categorized into three groups according to the findings of biopsy DKD, NDKD, and coexisting DKD with NDKD; the frequencies were 11 (18.30 %), 32 (53.30 %), and 17 (28.30 %), respectively. Amyloidosis is the most common renal disease, followed by acute tubular injury. Conclusion: This study demonstrated that NDKD is prevalent in T2DM, and renal biopsy is considered the gold standard for diagnosing renal pathology in diabetic patients.