Background: Inborn errors of metabolism (IEMs) are a group of congenital and chronic diseases that cause many difficulties for children and their parents. Objectives: This review aims to identify the needs that are faced in caring. Methods: This is an integrative review using the method of Whittemore and Knafl (2005). The databases of PubMed, Web of Science (ISI), SID, and Google Scholar were searched in English and Persian without any time and methodology restrictions in October and November 2022. Results: After screening the documents, 19 articles, consisting of 9 quantitative, 7 qualitative, and 3 review studies, were retrieved. Three categories of needs, including children, parents, and the healthcare system, were identified. The most reported needs were related to diet, psychological, social, and financial support dimensions and the necessity of implementing a multidisciplinary and coordinated care approach. Conclusions: Children with IEMs and their parents have many needs that can be satisfied through a multidisciplinary, patient-centered, and coordinated care approach.