Introduction : In Niger, as in the majority of countries south of the Sahara, malaria remains the major endemic disease and the first cause of morbidity and mortality in the most vulnerable groups, particularly children under 5 years of age. The objective of the study was to determine the epidemiological, clinical, therapeutic and evolutionary profile of children aged 0-59 months who died of severe malaria in the paediatric wards of the national hospital of Niamey. Method: This was a retrospective descriptive study conducted over a five-year period (1 January 2016 to 31 December 2020). Data were collected by non-probability sampling for convenience with exhaustive selection of all children's records meeting the selection criteria. The collected data were entered and analysed using SPSS version 23.0 software. Results: A total of 131 children died of severe malaria during the study period. The mean age of the deceased children was 28.18 (±16.86) months with extremes ranging from 1 to 59 months. Children who were less than or equal to 30 months of age were most affected in 57.30% of cases. The sex ratio was in favour of males in 59.50% of cases. The majority of the children (52.70%) came from an urban environment and 71.80% of the parents of the deceased children had a low socio-economic level. Clinically, 89.30% were referred to the national hospital by another health facility. The predominant signs of seriousness were lethargy (71.0%), altered consciousness (71%), severe pallor (57.30%) and convulsion (48.10%). The most frequent clinical forms observed in the deceased children were neurological malaria (41.20%), anaemia (28.20%), and mixed malaria (11.40%). On the therapeutic plan. Conclusion: Severe malaria is a public health priority in Niger and remains the leading cause of hospitalisation in the national hospital of Niamey with a lethality of 4%. A reinforcement of the technical platform, as well as preventive measures are necessary to reduce the death rate related to severe malaria in paediatric services.