World Health Organization defines oral health in terms of general, physical, psychological, and social well-being and teeth and gums speak a lot about the overall physiological well-being. The indication of our internal health can be examined through prominent changes in teeth, gums, or tongue. A similar indication has been seen in a case of tooth enamel defect related to renal disease called Nephrocalcinosis. Recently, a patient reported at City Dental Clinic with the chief complaint of delayed eruption of upper front teeth. Based on oral examination, a tooth development disorder of Amelogenesis Imperfecta was anticipated. However, the symptoms of generalized Gingival Fibromatosis increased the probability of an underlying Renal Syndrome. Renal ultrasound ascertained the existence of Bilateral Nephrocalcinosis, a rare genetic kidney disease, which thereby confirmed the diagnosis of Enamel-Renal Syndrome. Enamel formation or Amelogenesis is a systematic procedure of tooth enamel de