We report two boys, first aged 2.5 years and second aged 13 months. The first boy was hospitalized for the first time at the age of 8 mo due to generalized seizure. The other boy is now followed-up for 5 months, first time at the age of 8 mo had convulsive status epilepticus during fever. Brain MRI and interictal EEG in both children were normal. Other couses of seizures were excluded. The clinical progress in the first boy was marked by the development of focal seizures, with no unilateral domination, mainly in the form of status epilepticus triggered by fever. Ictal EEG showed paroxysmal brain activity correlated with clinical hemiconvulsions. He had pharmacoresistant epilepsy. Later, form of seizures has changed, with dominant atypical absence seizures. Psychomotor evaluation at the age of 2 years revealed mild deterioration. We performed genetic testing for Dravet syndrome. The mutation of SCN1A and SCN2 gene was negative. Analysis for GABRA1, GABRG2 and STXBP1 mutations is in progress. The clinical progress in second boy was marked by second status epilepticus during fever after we performed SCN1A gene mutation which is positive. The boy is now 13 months old, since now he had 6 episodes of status epilepticus, without other type of seizures. His interictal EEG and psychomotor development were normal. Clinical progress of the disease in the first boy is complient with Dravet syndrome and could be included among the 30% of patients without the mutation of SCN1A gene. Second boy has good clinical course after 5 mo follow-up, despite of the SCN1A gene mutation. Early diagnosis of Dravet syndrome is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. In both children remains an open question of using specific and/or preventive therapy with stiripentol as well as long-term prognosis. We report two boys, first aged 2.5 years and second aged 13 months. The first boy was hospitalized for the first time at the age of 8 mo due to generalized seizure. The other boy is now followed-up for 5 months, first time at the age of 8 mo had convulsive status epilepticus during fever. Brain MRI and interictal EEG in both children were normal. Other couses of seizures were excluded. The clinical progress in the first boy was marked by the development of focal seizures, with no unilateral domination, mainly in the form of status epilepticus triggered by fever. Ictal EEG showed paroxysmal brain activity correlated with clinical hemiconvulsions. He had pharmacoresistant epilepsy. Later, form of seizures has changed, with dominant atypical absence seizures. Psychomotor evaluation at the age of 2 years revealed mild deterioration. We performed genetic testing for Dravet syndrome. The mutation of SCN1A and SCN2 gene was negative. Analysis for GABRA1, GABRG2 and STXBP1 mutations is in progress. The clinical progress in second boy was marked by second status epilepticus during fever after we performed SCN1A gene mutation which is positive. The boy is now 13 months old, since now he had 6 episodes of status epilepticus, without other type of seizures. His interictal EEG and psychomotor development were normal. Clinical progress of the disease in the first boy is complient with Dravet syndrome and could be included among the 30% of patients without the mutation of SCN1A gene. Second boy has good clinical course after 5 mo follow-up, despite of the SCN1A gene mutation. Early diagnosis of Dravet syndrome is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. In both children remains an open question of using specific and/or preventive therapy with stiripentol as well as long-term prognosis.