Introduction: The hydatid cyst (HC) of the right ventricle (RV) is an extremely uncommon and a serious location that can cause sudden death following pulmonary embolism, obstruction of the valvular orifice or anaphylactic shock.
Case Presentation: We report a case of a 14 years-old girl with a HC of the RV. Surgical excision of the HC under Cardiopulmonary bypass (CPB) was successful in managing this rare case.