1. Two cases of congenital diabetes insipidus resistant to pitressin, or diabetes insipidus of the nephrogenic type, occurring in male cousins during infancy have been described in which the most striking manifestations were recurrent pyrexia, polyuria, polydipsia, poor weight gain and development and hyperelectrolytemia.2. The basic defect in these patients appears to be renal; in the nature of an endorgan failure to respond to the antidiuretic hormone of the posterior pituitary body.3. The demonstration of the trait in the mothers of the patients and probably an uncle and maternal grandmother suggests genetic transmission by means other than the previously postulated sex-linked recessive pattern.4. The literature pertinent to this condition is reviewed and some of the clinical features and diagnostic problems are discussed.
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