['Proteinuria suggests kidney involvement in Fabry disease. We assessed podocyturia, an early biomarker, in controls and patients with and without enzyme therapy, correlating podocyturia with proteinuria and renal function.', 'Cross-sectional study (n\xa0=\xa067): controls (Group 1, n\xa0=\xa030) vs. Fabry disease (Group 2, n\xa0=\xa037) subdivided into untreated (2A, n\xa0=\xa019) and treated (2B, n\xa0=\xa018). Variables evaluated: age, gender, creatinine, CKD-EPI, proteinuria, podocyte count/10 20× microscopy power fields, podocytes/100\xa0ml urine, podocytes/g creatininuria (results expressed as median and range).', 'Group 1 vs. 2 did not differ concerning age, gender and CKD-EPI, but differed regarding proteinuria and podocyturia. Group 2A vs. 2B: age: 29 (18-74) vs. 43 (18-65) years (p\xa0=\xa0ns); gender: males n\xa0=\xa03 (16\xa0%) vs. n\xa0=\xa09 (50\xa0%). Proteinuria was significantly higher in Fabry treated patients, while CKD-EPI and podocyturia were significantly elevated in untreated individuals. Significant correlations: group 2A: age-proteinuria, ρ\xa0=\xa00.62 (p\xa0=\xa00.0044); age-CKD-EPI, ρ\xa0=\xa0-0.84 (p\xa0<\xa00.0001); podocyturia-podocytes/100\xa0ml urine, ρ\xa0=\xa00.99 (p\xa0=\xa00.0001); podocyturia-podocytes/g creatininuria ρ\xa0=\xa00.86 (p\xa0=\xa00.0003), podocytes/100\xa0ml urine-podocytes/g urinary creatinine, ρ\xa0=\xa00.84 (p\xa0=\xa00.0004); proteinuria-CKD-EPI, ρ\xa0=\xa0-0.68 (p\xa0=\xa00.0013). Group 2B: podocyturia-podocytes/100\xa0ml urine, ρ\xa0=\xa00.88 (p\xa0<\xa00.0001); podocyturia-podocytes/g creatininuria, ρ\xa0=\xa00.84 (p\xa0<\xa00.0001); podocytes/100\xa0ml urine-podocytes/g creatininuria, ρ\xa0=\xa00.94 (p\xa0<\xa00.0001); CKD-EPI-proteinuria, ρ\xa0=\xa0-0.66 (p\xa0=\xa00.0028).'] Is podocyturia significantly elevated in untreated vs treated Fabry adult patients?