Aim To identify, analyse, and synthesise existing research on the characteristics and risk factors associated with primary immune deficiencies (PIDs), with focus on understanding how factors impede patient outcomes. Background There is currently limited research regarding the management of this cohort when they present to an emergency department with the presentation urgency often being overlooked. Method Three databases, google scholar, and citations were searched for relevant studies under the criteria. Included papers were analysed and reported following the PRISMA guideline, and then critically appraised using the Mixed Method Appraisal Tool. Results After a review of 625 titles and abstracts, 20 studies met the inclusion criteria. The majority being mixed method (n = 8) and case studies (n = 8). All chosen studies reported some form of management of a child with a PID, and most made recommendations for improvement. Conclusions Further research is needed to facilitate an understanding of how to enhance emergency management, to increase positive outcomes. Relevance to practice: There is a critical need for improved management strategies for children with a PID presenting to ED with fever. Creating protocols, increasing staff knowledge, and implementing patient specific interventions are essential in improving outcomes and reducing serious complications in this high-risk paediatric population.