Cystic hydatid disease commonly affects the liver and lungs. Cardiac hydatid cysts are a rare occurrence and can cause fatal complications, including anaphylactic shock, systemic or pulmonary embolism, dissemination, arrhythmias, valvular dysfunction or sudden death. The case of a 19-year-old male from a rural area who was admitted to the emergency room with anaphylactic shock is presented in the current study. The patient was subsequently referred to the Department of Pneumology due to a mild fever, a dry cough and thoracic pain. Pneumonia was suspected and antibiotics were administered. As the patient didn't respond to the antibiotics, a pulmonary CT-scan was performed, which demonstrated a bilateral pulmonary embolism of the segmental arteries and the patient was referred to the Department of Cardiology. Transthoracic echocardiography (TTE) demonstrated the presence of a right ventricular mass of ~25x18 mm, attached to its free wall, adjacent to the tricuspid valve causing no tricuspid inflow obstruction. Blood tests were positive for echinococcal infection. Considering that previous reviews had included cases published up until 2018, the present study also included a short literature review of the studies published between 2018 and 2021. The review showed that cardiac hydatid cysts are diagnosed more often in underdeveloped countries, especially in men. Transthoracic echocardiography is the most useful non-invasive imaging technique for diagnosis. Surgery is the treatment of choice, but consideration must be given to the risk-benefit ratio and the shared decision-making approach. The complete surgical removal of the cyst(s) is the major prognosis factor of the cardiac manifestation. This study emphasizes the importance of considering cardiac echinococcosis as a potential diagnosis in patients from endemic or farming areas.