AimsChildren with a congenital upper limb difference (CoULD) are a diverse group who often require multidisciplinary care and long‐term support for functional and social impacts. The Australian Hand Difference Register (AHDR) provides a national database of children born with a CoULD and aims to facilitate research and improve health care for affected children. Using data from the first 3 years of its operation, we analysed the demographic and clinical features of participating families, including type of CoULDs and the frequency of pre‐natal and syndromic diagnoses.MethodsFamilies were recruited from tertiary plastic surgery, orthopaedic and genetics clinics, as well as by self‐referral. Hand differences were classified by the consulting physician according to the Oberg–Manske–Tonkin classification system. Primary carers were invited to complete an online questionnaire covering demographic information, pregnancy and newborn outcomes and diagnostic details.ResultsBetween August 2017 and September 2020, 822 families consented and 320 questionnaires were reviewed. CoULDs were detected pre‐natally in 66 (20.6%) and post‐natally in 248 children (77.5%); data for 6 (1.9%) children were missing. The most common CoULDs were radial polydactyly, symbrachydactyly with ectodermal elements and radial longitudinal deficiency, hypoplastic thumb. Twenty‐seven children (8.4%) had an associated syndrome, 7 diagnosed pre‐natally and 19 post‐natally; the most common were VACTERL association, Poland anomaly, Holt–Oram and ectrodactyly–ectodermal dysplasia–clefting syndromes.ConclusionsThe AHDR is a valuable resource for understanding the relative frequencies of CoULDs. Participation will assist future research into the diagnostic journeys of children with CoULDs, including risk factors, diagnosis and psychosocial impacts.