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Background: Pericardial tumors have a rare occurrence. The incidence of pericardial tumors is 6.7 to 12.8% of all cardiac tumors, which in turn accounts for only 0.001 to 0.03% of all malignancies. Lymphangiohemangiomas are tumors which can occur at any age group, in any part of the body. Lymphangiohemangioma of the pericardium is a rare phenomenon. Here we present an extremely rare case of pediatric pericardial lymphangiohemangioma presenting with massive pericardial effusion resulting in cardiac tamponade uniquely managed by subtotal pericardiectomy followed by administration of mTOR inhibitor based on the histopathology and PET scan. Case Presentation: A 6-year-old male child, 2nd twin of 3rddegree consanguineous marriage presented to the emergency department and was admitted in Pediatric Intensive Care Unit with complaints of progressive difficulty in breathing since 3 months cough and rhinorrhea since 2 days and chest pain since 2 days. His birth history, immunization and developmental history were not significant for the present complaints, Anthropometry was within normal range. Outcome and follow up: Postoperatively the patient improved gradually. Intercostal drains were removed and patient was shifted out of the ICU on O2 support with nasal prongs. However, he continued to have respiratory distress. Ultrasonography chest revealed consolidation and bilateral hemorrhagic pleural effusion. Repeat ultrasonographys showed increased pleural effusion. His respiratory distress increased so he was put onmechanical ventilation and Ultrasonography guided pleural tapping was done thrice. After starting Sirolimus, gradually, condition of the child improved, pleural effusion reduced, and he was shifted to the ward on High Flow Nasal Cannula. Conclusion: To our knowledge, this is the only reported case of diffuse pericardial lymphangiohematomatosis. In this particular case, subtotal pericardiectomy resulted in symptomatic relief by relieving the cardiac tamponade and resultant cardiac compression. This approach was preferred in this case over the other option of pericardial window creation taking into account the need for complete drainage of the massive pericardial tamponade, removal of adequate pericardial tissue which was acting as the source of pericardial effusion and acquiring adequate and representative tissue for histopathological diagnosis of the disease. [ABSTRACT FROM AUTHOR]