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She died of hepatic failure 9 mo after hepatic MUP was diagnosed. This the first case of hepatic MUP treated with combined ipilimumab and nivolumab, who showed better outcome than previous cases. Combined ICIs of PD-1 and CTLA-4 may be considered as the first-line therapy for patients with hepatic MUP. Combined ICIs of PD-1 and CTLA-4 may be considered as the first-line therapy for patients with hepatic MUP. Double outlet right ventricle (DORV) is a rare and complex congenital heart defect, and the surgical repairs vary with type and p