Congenital renal arteriovenous malformations are very rare benign lesions. They are more common in women and rarely manifest in elderly people. In some cases, they present with massive hematuria. Contemporary treatment consists of transcatheter selective arterial embolization which leads to resolution of the hematuria while preserving renal parenchyma. Despite the emergence of the minimally invasive procedures, open surgery in the form of partial nephrectomy or nephrectomy remain viable options. This paper aimed to report a rare case of congenital renal arteriovenous malformation, its clinical manifestations and the surgical treatment chosen.