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A hyperosmolar hyperglycemic state (HHS) is a rare presentation of a hyperglycemic crisis in children with diabetes mellitus. As this condition can be fatal and has high morbidity, early recognition and proper management are necessary for a better outcome. Here, we report a rare case of HHS as the first presentation of type 1 diabetes mellitus (T1DM) in a 7-year-old girl.The patient was admitted due to polyuria and weight loss in the past few days. The initial blood glucose level was 1167mg/dL.On the basis of clinical manifestations and laboratory results, she was diagnosed with T1DM and HHS.Treatment was started with intravenous fluid and regular insulin.She was discharged without any complications related to HHS and is being followed up in the outpatient clinic with split insulin therapy.As the incidence of T1DM is increasing, emergency physicians and pediatricians should be aware of HHS to make an early diagnosis for appropriate management, as it can be complicated in young children with T1DM.