Supernumerary teeth (SN) teeth are a relatively rare phenomenon that can occur anywhere in the dental arch. This report represents a case of a young female patient that is presented with one SN tooth specifically mesiodens, which prevented her upper central incisor from erupting in its normal sequence. This patient also reported to have a congenital complete duplex of left kidney that has much smaller upper pole and a normal size lower one, with a sperate ureter of each, and growth hormone deficiency. This is a non-syndromic patient. In the literature review there has been some linking between growth hormone deficiency and SN teeth. However, up to this research in the literature, no case was found linking between complete duplex of kidney and ureter, and SN tooth. This report also viewing radiographic CBCT regarding mesiodens, in three views (coronal, sagittal, and axial).