Objective To report the demographic, clinical and paraclinical characteristics as well as the outcomes during follow-up visits of HHE syndrome in Niger. Patients and methods It was a retrospective study conducted at the Neurology Outpatient Clinic of the National Hospital of Niamey (Niger) between May 2003 and May 2018. Results During the period of study, 882 patients with epilepsy aged 20 years or less were seen in consultation among whom we collected 22 cases of HHE syndrome with a hospital prevalence of 2.5%. They were 14 men and 8 women (sex ratio = 1.75) with a mean age of 3.76 years (range: 1 and 20 years). At the time of diagnosis, 81.8% of patients were aged under 6 years. The antecedent of febrile convulsions in childhood was found in only 31.8% of cases. The mean age of onset of hemiplegia was 1.9 years (range: 9 months and 5 years). The mean age of onset of epileptic seizures was 2.94 years (range: 1 and 8 years). The mean interval between the onset of hemiplegia and onset of epileptic seizures was 9 months (range: 1 month and 4 years). 31.8% of patients had a delay of acquisitions at the time of diagnosis. 95.5% of patients had drug-resistant epilepsy. Conclusion HHE syndrome is still seen in the countries of Sub-Saharan Africa. The high morbidity and mortality in children with HHE syndrome highlight the need to improve emergency care for febrile clonic seizures in childhood and the early and adequate management of infectious diseases in the child.