To outline the epidemiology and clinical pathway of acute rheumatic fever (ARF) cases in the Waikato District Health Board (DHB) region of New Zealand. An audit was carried out of the clinical notes of all recognised ARF cases from 1998 to 2004 (inclusive) residing within the Waikato DHB region at diagnosis. Cases were identified by using the hospital admissions coding system and the EpiSurv notification system. The case definition used was the New Zealand criteria for ARF diagnosis, which includes echocardiographic evidence of carditis as a major criteria. A total of 77 ARF cases were found, 8 of which were recurrences. An annual rate of 3.0 per 100,000 initial cases or 3.3 per 100,000 population (initial and recurrent cases) was documented. Over 80% of the total initial ARF cases in the Waikato DHB were in the 5-14 year age group. The overall annual incidence in this age group was 12.9 per 100,000 (age specific incidence for Maori aged 5-14 years 39.6 per 100,000 and for NZ European aged 5-14 years 2 per 100,000). The majority of cases found were Māori (83%), and residing in low socioeconomic status (80% living at the time of diagnosis within the most deprived three deciles according to NZDep01). The presence of 77 cases in the Waikato DHB region from 1998-2004 compares unfavourably with other regions, and implies a significant burden from this disease. ARF is a preventable chronic disease with potential life-long sequelae. If the rate of ARF in Māori was reduced to that of non-Maori non-Pacific, then the burden of this disease to New Zealand communities and to the health sector would be virtually eliminated and inequalities improved.