BACKGROUND: Calcium oxalate (CaOx) nephropathy, from primary hyperoxalosis (PH) or secondary hyperoxalosis (SH), is a very rare but potentially devastating disease predisposing to end-stage renal disease (ESRD) and the diagnosis is often delayed. There is also a paucity of studies documenting their spectrum of clinical features in longitudinal practice. We aimed to investigate the clinical presentation, urinalysis, renal function, urine biochemistry, kidney biopsy findings, and renal outcomes in our hospital, to see their characteristics comparing documented literature and some probable correlations or clues for prompt diagnosis between its two forms. METHODS: This retrospective, observational cohort study which was conducted at Tri-Service General Hospital, National Defense Medical Center from 2011 to 2021. Patients who had undergone kidney biopsy with pathologically proven CaOx crystals deposition and nephropathy were included. Clinical profiles and possible etiologies were obtained from review of clinical charts. We in advanced arranged descriptive statistics presented in the median for non-normal distribution and Spearman's correlation analysis for some characteristic data. RESULTS: Six individuals with CaOx nephropathy were identified. The median age of onset was 52 (21-78) years old, the male was predominant (83.3%) and median age on renal replacement therapy (RRT) was 48 (21-55) years old in the subgroup with ESRD. The median body height 169 (158-177) cm was lower than the mean male height in Taiwan (174.52 cm, 1996). Common presentations contained renal failure (100%, 6/6), pyuria (66.7%, 4/6), hematuria (66.7%, 4/6), lower grade proteinuria (83.3%), and non-crystalluria (100/%, 6/6) but none were specific. Spearman's correlation analysis showed concentration of serum inorganic phosphate positively correlated with estimated glomerular filtration rate (eGFR) decline rate before kidney biopsy (pre-bx GFR rate) (correlation coefficient, 0.829; P = 0.042). Concentration of urine phosphate and urine specific gravity nearby pre-kidney biopsy date correlated negatively (correlation coefficient, -0.900, -0.943; P = 0.0.37, 0.005, respectively). Histopathological findings consisted with pre-bx GFR rate and renal outcome. In the grouped compartment, two cases who had no hyperoxaluria-enabling conditions had the highest possibility to the diagnosis of PH, presented with most severity of histological crystal nephropathy, positive prior nephrolithiasis/nephrocalcinosis, largest pre-bx GFR rate, and dependence on RRT. Some comorbidities or trigger factors should be identified and prevented clinically. We finally proposed an approach flow chart to help prompt diagnosis and management for PH and SH. CONCLUSION: Detailed history taking, biochemistry, and pathologic characteristics provided value for prompt diagnosis and adequate management of oxalate nephropathy.