The syndrome of hyperosmolar diabetic coma has been well described in adults but rarely documented in children. Six children, not previously known to have diabetes mellitus, have been observed with this syndrome. The patients presented with extreme dehydration and coma following brief, nonspecific illnesses. Pre-existing neurological damage was present in 4, including 2 with Down's syndrome. There was a family history of diabetes mellitus in 4 children. The biochemical picture was characterized by hyperglycemia, hyperosmolality, metabolic acidosis, and minimal or no ketosis. Death occurred in 4 patients. Two children survived, one without neurological sequelae and the other with significant brain damage. Only those patients receiving minimal glucose in the initial parenteral solution survived. Hyperosmolality may be a significant factor in diabetic deaths in children. © 1969 The C. V. Mosby Company.