During a child’s development, one of the key milestones is tooth eruption, which typically begins between 6 and 8 months of age. Depending on genetic, endocrine, and environmental factors, this process can vary in time. One of such alternatives is natal teeth – the presence of teeth at birth. The etiology of natal teeth remains unknown, with only certain hypotheses. The literature describes numerous genetic syndromes associated with natal teeth, but there is no specific syndrome that combines only natal teeth and unilateral renal agenesis. We present such a unique case in Armenia, where a multidisciplinary and continuous approach helped to prevent potential complications in a child.