Heart transplantation (HTx) is a gold standard for end-stage heart failure (ESHF). Cardiomyopathies form the majority of patients who undergo HTx. Grown-up congenital heart disease, with or without prior palliative surgery, progresses to ESHF requiring HTx. They constitute the least among all heart recipients. The immediate posttransplant management may become challenging due to severe right heart failure, massive ascites, pleural effusion, and cardiac cachexia. Scarce data are available on this subset of patients. We describe one such patient's post-HTx management. A 15-year-old male with Ebstein's anomaly, restrictive cardiomyopathy, severe right ventricular (RV) dysfunction, atrial flutter, left atrial clot, postradiofrequency ablation, New York Heart Association III, and early cardiac cirrhosis underwent HTx successfully. He had significant mediastinal hemorrhage postoperatively, and managed medically; echocardiography showed a good biventricular function with mild tricuspid regurgitation (TR), and he was extubated on the 1st postoperative day. However, progressive RV function deterioration was observed over the next 72 h (tricuspid annular plane systolic excursion of 7 mm with mild TR) with massive worsening ascites and pleural effusion. He was treated with milrinone and furosemide infusion and noninvasive ventilation, but response to optimal diuretic doses was poor, and the ascites did not decrease. Hence, intermittent paracentesis was done, and 10 L of ascitic fluid was removed over 10 days. Then, he responded to diuretics; his RV function improved. His respiratory support and inotropes were discontinued. His immunosuppressants consisted of tacrolimus and prednisolone. Mycophenolate mofetil was withheld due to leukopenia. At 6-month follow-up, his cardiac functions were normal, ascites completely resolved, appetite improved, and he gained weight.