Method: A 21-year-old male was referred to nephrology with a 3 months history of intermittent gross haematuria. All blood test including blood cell count, biochemistry, immunoglobulin electrophoresis, antinuclear antibody were normal. Urinary analysis revealed numerous red blood cells and a protein/creatinine ratio of 0.3. A previous cystoscopic examination was normal. Renal ultrasound revealed a diffuse increased echogenicity. Results: Renal biopsy showed a normal histology with no immunofluorescence deposits. CT scan revealed dilatation of the distal left renal vein with narrowing between the superior mesenteric artery and the aorta. Conclusion: NS is a rare entity causing haematuria. There are few reported cases with histology, although it usually shows no abnormalities. The proposed mechanism for the haematuria is an abnormal communication between the submucosal venous plexus and the calyceal system presumably induced by renal venous hypertension.