Introduction: Rare vascular abnormalities called renal arteriovenous malformations (AVMs) can cause hematuria among other symptoms. Due to overlapping clinical and radiological symptoms, differentiating between renal AVMs and other disorders such renal cell carcinoma (RCC) can be difficult. Here, we report a case of a patient who developed hematuria and was first diagnosed with renal AVM. We go over the difficulties in diagnosing this ailment and the approaches to managing it. Case Presentation: A 34-year-old female patient, reported having hematuria with clots for one week, coupled with fever and left loin discomfort that persisted for four and one day, respectively. Her hemoglobin level was 4.5 g/dL at arrival, which required packed red cell transfusions. A left double-J stent was implanted after an emergency cystoscopy revealed bloody efflux from the left ureteric orifice. A potential arteriovenous malformation in the left kidney's upper pole area was discovered by further imaging. A cirsoid-type AV abnormality arising from the superior polar branch of the renal artery was verified by renal angiography. After therapeutic embolization, the patient's symptoms were relieved and hematuria stopped. Discussion: The case study highlights the need of taking renal AVMs into account when making a differential diagnosis for hematuria, especially if there haven't been any previous urological operations. It also emphasises how important imaging techniques like renal angiography and renal ultrasonography with Doppler are for making the right diagnosis. Distinctive diagnosis from other ailments, such as renal cell carcinoma, highlights the necessity of comprehensive assessment and monitoring to guarantee suitable treatment. Conclusion: Renal arteriovenous malformations are rare but important causes of hematuria. Prompt diagnosis and management are crucial to prevent complications and improve patient outcomes. This case report demonstrates the diagnostic challenges associated with renal AVMs and underscores the importance of a multidisciplinary approach in their evaluation and treatment.
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