We used statewide surveillance data to describe the epidemiology of invasive Haemophilus influenzae type a (Hia) disease in Alaska during 2018-2022. Of 52 cases identified, 39 (75%) occurred among Alaska Native children aged <
5 years who lived in rural areas of southwest or northern Alaska. Average annual incidence was 17.8 per 100,000 among children aged <
5 years compared to 0.3 per 100,000 among persons aged ≥5 years. Among 43 cases in children aged <
5 years, 16 (37%) presented with meningitis and 6 (14%) died. Characterizing Hia disease epidemiology can help direct prevention strategies, including vaccine development and use.