Background Cystic Hydatid disease is a parasitic infection with a worldwide distribution. It is caused by the larval stages of a species of tapeworms known as Echinococcus granulosus. Even in endemic areas; Cardiac involvement by hydatidiosis is very rare and has atypical presentations as well as localization which make it undiagnosed in about 10% of cases. The left ventricle is the most Common chamber involved by the hydatid cyst and isolated involvement of the right ventricle is very rare, especially in children. The aim of the present study was to describe hydatid cardiac cyst of the right ventricle of a child. Case presentation We present a rare case of an 8 year-old boy, living in a rural area, who was diagnosed with a cardiac hydatid cyst in the right ventricle. He also had multiple pulmonary hydatid cysts and presented with dyspnea, cough and atypical chest pain. The patient underwent surgery for the resection of pulmonary cysts and, subsequently, cardiac hydatid cyst. The outcome was favorable seven weeks after surgery and there was no clinical and echocardiographic recurrence. Conclusion Cardiac Echinococcosis must be suspected in endemic areas, diagnosed with appropriate imaging techniques, and treated appropriately.