Aims To identify, analyse, and synthesise retrospective data regarding the characteristics and risk factors that primary immune deficiencies (PIDs) inhibit to enhance patient outcomes and improve healthcare professional knowledge. Background There is currently limited research regarding the management of this high-risk paediatric cohort when they present to an emergency department (ED). This review analyses clinical data in the management, treatment and outcomes for these patients. Design This retrospective cohort review analysed patient characteristics, including the ED presentation and treatments, and hospital outcomes for children with a PID. Method Data from electronic medical records were extracted at a large tertiary paediatric hospital in South-East Queensland according to inclusion and exclusion criteria. Identified cases deidentified, analysed and reported. Baseline variables summarised using descriptive statistics. Results Out of 789 ED presentations relating either to fever or PID, 126 cases met the inclusion criteria. Overall, the length of time to be seen by a clinician in ED was a mean of 83 min, 27.8 % did not receive any treatment. Eleven patients had no investigations performed at all, of those who did, 5 % returned positive blood cultures. The immunology team were not consulted for 52 % of patients, and among those admitted, 70.3 % were classed as having a complex medical history. Conclusions There is limited consistency surrounding the management of children with a PID who present to the ED with a fever. Further research and resources are needed to facilitate enhanced emergency management to increase positive outcomes for this rare, but at-risk cohort of paediatric patients.