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• To identify the similarities and differences in the guidelines for CRMS from Cystic Fibrosis Foundation and from European Cystic Fibrosis Society. • To understand the diagnostic challenges for CRMS in patients with prenatally identified CFTR variants. • To identify the various sources of information for classifying CFTR variants as disease causing and how these assignments may change over time. • To recognize and monitor for the possibility of progression over time from a diagnosis of CRMS to CF in a subset of patients with CRMS. • To identify gaps in the guidelines that may not cover every clinical scenario that is encountered during the long-term follow up of patients with CRMS. Newborn screening (NBS) for cystic fibrosis (CF) has enabled earlier diagnosis and has improved nutritional and growth-related outcomes in children with CF. For those with a positive NBS for CF that do not meet the diagnostic criteria for CF, the clinical entity called CFTR-Related Metabolic Syndrome (CRMS) or CF Screen- Positive, Inconclusive Diagnosis (CFSPID) is used. Although most children with CRMS remain relatively asymptomatic, studies have shown that between 11% and 48% of these patients may eventually progress to a diagnosis of CF over time. Although the CF Foundation guidelines for CRMS management and European CF Society guidelines for CFSPID have some similarities, there are also some differences. Here, we review challenging case scenarios that highlight remaining gaps in CRMS guidelines, thus supporting the need to update and unify existing guidelines. [ABSTRACT FROM AUTHOR]