We present the case of a 3-year-old female with cyclic neutropenia and history of febrile seizures who died acutely from coronavirus disease 2019 (COVID-19). While most children with COVID-19 infection are asymptomatic or have mild symptoms, there may be increased risk of severe disease in immunocompromised children. To the best of our knowledge, this is the first reported case of a COVID-19-related death in a pediatric patient with cyclic neutropenia.