Background Aboriginal leaders invited us to examine the frequency and reasons for children presenting to Fitzroy Crossing Hospital emergency department (ED), Western Australia.Methods ED presentations (2007-11 inclusive) were examined for children born in the Fitzroy Valley in 2002-03.Results ED data were examined for 127/134 (94.7%) eligible children, with 1058 presentations for 1743 conditions in children aged 3-9 over 5 years. Most (81%) had at least one ED presentation (median 9.0, range 1-50). There were no differences by sex or season but numbers of presentations increased over time. Common presentations included: injury (15.1%), diseases of the ear (14.9%), skin (13.8%), respiratory tract (13.4%), infectious and parasitic diseases (9.8%), screening, follow up, and social reasons (16.0%). Household over-crowding, financial and food insecurity were common socio-economic factors recorded at ED presentation. Prenatal alcohol exposure (PAE) was significantly associated with high-frequency (>10) ED presentations.Conclusion Children in very remote Fitzroy Crossing communities have high rates of preventable ED presentations, with higher rates in those with than without PAE. Support for culturally appropriate preventative programs and improved access to health services need to be provided in remote Australia.