The impact of hemophilia in affected women and girls (WG) is insufficiently recognized, resulting in sub-optimal evaluation of bleeding symptoms, delay in diagnosis and missed management opportunities. The aim of this collaborative International Society on Thrombosis and Haemostasis (ISTH) Scientific Subcommittee (SSC) on Pediatric and Neonatal Thrombosis and Haemostasis, Factor VIII, Factor IX and Rare Coagulation Disorders, and Women's Health Issues in Thrombosis and Haemostasis project is to provide guidance recommendations regarding screening and diagnosis of hemophilia carriers (HC)/WG with hemophilia (WGwH) in addition to the previously published revised nomenclature by the SSC of the ISTH. We provide several guidance recommendations regarding the nomenclature, utilization of the ISTH-Bleeding Assessment Tool (ISTH-BAT) as a tool to quantify bleeding phenotype, factor VIII/IX assays for testing, assay discrepancy, genetic testing and testing during pregnancy and postpartum, to help guide hemophilia providers to streamline the screening and diagnosis of HC/WGwH in a uniform manner. We hope, once the screening and diagnosis of HC/WGwH becomes a uniform standard practice globally, this will then pave the way for global harmonization of terminologies, improved management, bringing gender equity in hemophilia care a step forward.