We present a new clinical case of a 25-year-old man with Wolfram's syndrome. Partial central diabetes insipidus was found to cause polyuria, polydipsia and enuresis. The patient had diabetes mellitus for the last 18 years, controlled with diet and/or chlorpropamide during 10 years and showed no typical complications of the disease. The patient was also shown to have a dilated urinary tract and perceptive hypoacusis for high frequencies. The fifth classical component of this syndrome, primary optic atrophy, was not found. Based on the report of this case, Wolfram's syndrome is briefly described.