Background The importance of clinical presentation and pre-transplantation course on outcome in children with dilated cardiomyopathy (DCM) listed for heart transplantation is not well defined. Methods and Results The impact of age, duration of illness, gender, race, ventricular geometry and the diagnosis of myocarditis on outcome in 261 DCM children enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. Endpoints included: 1) listing as UNOS Status 1, 2) death while waiting and 3) death post-transplantation. The median age at the time of diagnosis was 3.4 years, and mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (p=0.0006 and p=0.03, respectively). Shorter duration of illness (p=0.04) was associated with listing as UNOS Status 1. Death post-transplantation was associated with myocarditis at presentation (p=0.009), non-white race (p<0.0001) and a lower left ventricular end-diastolic dimension z-score at presentation (p=0.04). In the myocarditis group, 17% (4/23) died of acute rejection post-transplantation. Conclusions Mechanical ventilator use and older age at listing predicted death while waiting, while non-white race, smaller left ventricular dimension and myocarditis were associated with death post-transplantation. Although 97% of children with clinically or biopsy diagnosed myocarditis at presentation survived to transplantation, they had significantly higher mortality post-transplantation compared with children without myocarditis, raising the possibility that pre-existing viral infection or inflammation adversely affects graft survival.