INTRODUCTION AND IMPORTANCE: Retrocaval ureter is a rare congenital abnormality resulting from abnormal development of the inferior vena cava (IVC), leading to compression and obstruction of the right ureter. The condition is frequently diagnosed incidentally or presents symptoms related to hydronephrosis. The left retroaortic renal vein is another vascular anomaly with an estimated prevalence of 1.7 % to 3.4 %. However, it is typically asymptomatic. CASE PRESENTATION: We present the case of a 9-year-old boy with a history of undescended right testis and hypospadias who presented with intermittent pain and hematuria. Physical examination revealed mild tenderness of the renal angle. Imaging studies, including ultrasound and uroscan, demonstrated right hydronephrosis, an S-shaped deformity of the proximal right ureter, and a medium calyceal stone. Interestingly, a left retroaortic renal vein was also identified. The patient underwent surgical correction using a conventional open approach, where the retrocaval segment was excised, excised, and an end-to-end uretero-ureteral anastomosis was performed on a double J stent. Postoperative recovery was uneventful and uneventful and follow-up imaging showed resolution of hydronephrosis. CLINICAL DISCUSSION: Retrocaval ureter is a developmental anomaly with an estimated prevalence of 0.13 %. It often presents urinary obstruction and recurrent infections. The diagnosis is based on imaging modalities, including 3D reconstruction CT urography. Treatment is recommended for symptomatic cases to prevent long-term renal deterioration. Surgical options include open, laparoscopic, and robotic approaches. In our case, conventional surgery was chosen due to the available anatomy and surgical expertise. CONCLUSION: Congenital anomalies of the IVC and renal veins, although rare, should be considered in paediatric patients with urinary compression. Advances in imaging have improved the diagnosis of these anomalies, allowing for timely and effective surgical management.