Traditionally, computed tomography angiography has been considered the gold standard for diagnosing renal arteriovenous malformation (AVM). Despite the sensitivity of ultrasound in detecting AVM through Doppler techniques, it often lacks the ability to provide detailed insights into the lesion. In this narrative, we present a case of unexplained hematuria accompanied by flank pain, which was conclusively diagnosed as congenital renal AVM via multimodal ultrasound. The pivotal contributions of color Doppler ultrasound and contrast-enhanced ultrasound are that they are instrumental in enabling an early and markedly precise diagnostic pathway.