Background: seizures are the most common neurologic emergency in pediatrics and can be terrifying for patients and families. Aim of the work: this study aimed to determine the etiologies and sex distribution of seizure in children admitted to Emergency Department of Maternity and Children's Hospital in Arar city, Northern Saudi Arabia, during the period from 1 January to 30 July 2017.  Methods: this was a descriptive hospital-based study conducted in the Department of Pediatrics Emergency, Maternal and Child Hospital of Arar City during the period from 1 January to 30 July 2017.  All the children below 12 years of age who were hospitalized at emergency department were enrolled in this study. The final diagnosis of seizure was made by a pediatric neurologist. The age, sex, type of seizure, associated fever, history of head trauma and other variables related to seizure in pediatric group were reviewed from the medical records. Results: a total of 158 children with diagnosis of seizures were included in this study. The proportion of male to female was about 2:1 as the percentage of males was 65.8% of patients. All patients up to 12 years were included with a mean age of 26.3±15.8 months. Less than quarter (16.5%) of the patients had focal seizures, while 83.5% had generalized seizures. Family history of seizures disorders were noted in only 15.2% patients. A previous history of seizure was mentioned in 19% patients. Febrile seizure was the most common etiology (72.2% of patients).  Epilepsy in 17.7% and vaccine-associated seizure in 5.1% of the patients. Hypoglycemia, intracranial hemorrhage, encephalitis and hyponatremia were reported 2(1.3%) for each of them. Conclusion and recommendations: as this is the first study conducted in Arar maternity and children's hospital to differentiate of the etiology of seizure in children attending the emergency department. Indeed, we suggested other researchers to follow the patients to show the repetitions of seizure and the prognosis in them especially those with dangerous etiologies as epilepsy, diabetes, encephalitis and intracranial hemorrhage. Fever coexistence, seizure type, physical and neurological examinations and history of head injury and measurement of serum blood sugar level may provide important information for primary emergency physicians when evaluating children with attack of seizures.