Diabetes insipidus (DI) is a condition characterized by excretion of large volumes of dilute urine secondary to either a deficiency in the production/release of the hormone arginine vasopressin (AVP) that is synthesized in the hypothalamus and transported and stored in the posterior pituitary for release in response to a rise in plasma concentrations of osmotically active substances or an impaired response or resistance to AVP at the level of the kidney (nephrogenic DI, 4). Here, AVP is linked to dentinogenesis imperfecta.