Introduction: In children, the association of ipsilateral pyeloureteral junction obstruction (PUJO) and ureterovesical junction obstruction (UVJO) is a rare malformation with a non-standardized treatment. We report a case of PUJO and UVJO treated by a combined minimally invasive surgical treatment to resolve the double urinary obstruction. The current literature was also reviewed. Case report and review: A two-month-old boy, without antenatal and postnatal signs of urinary tract anomalies, was hospitalized presenting right hydronephrosis, perirenal fluid effusion, and ascites. An acute pelvic rupture was suspected, and a retrograde pyelogram was performed, showing a primary obstructive megaureter (POM) associated with a corkscrew pyeloureteral junction. The impossibility to place a double J catheter through the pyeloureteral junction led us to achieve percutaneous nephrostomy and an abdominal drain placement. Three months later, the patient underwent a combined high-pressure balloon ureterovesical junction dilation and retroperitoneoscopic Anderson Hynes one-trocar-assisted pyeloplasty (OTAP). The literature search identified 110 children experiencing double urinary tract obstruction. All authors agreed on the difficulty to diagnose both obstructions preoperatively, but there is still no consensus on which obstruction should be relieved earlier, because the alteration in urinary vascularity during a double surgery could damage the ureter. Conclusions: The simultaneous occurrence of UPJO and UVJO is rare, with a challenging diagnosis. Prompt identification and timely surgical intervention are crucial to mitigate the risk of renal function loss attributable to obstruction and infection. Drawing from our expertise and the analysis of the existing literature, we propose employing a simultaneous double minimally invasive strategy in order to optimize the preservation of ureteral vascularity. This approach entails performing a minimally invasive pyeloplasty for the PUJ and utilizing high-pressure balloon dilatation for the UVJ.