The management of children with organic acidemias (OAs) is limited in nursing literature. This article focuses on the two more common OAs, methylmalonic and propionic acidemias. Literature search was done on PUBMED, CINAHL, OVID, UptoDate, and GeneReview. The benefit of early diagnosis and treatment has been well documented, but many unresolved aspects of care management remain. Patient care is a complex necessitation and a lifelong follow-up for complications. Caring for patients with OA requires that nurses increase their familiarity with metabolic genetics and develop a better understanding of proper medical and nursing management while research continues to determine the most beneficial treatment and long-term care management methods.