Introduction: Primary cardiac tumors are uncommon during infancy and childhood. Myxomas originating from the right ventricle are even less common in pediatric patients.
Case presentation: Here we describe a case of an 11-year-old Tunisian boy who was referred for syncope. Transthoracic echocardiography revealed a large mobile mass attached to his right ventricle, obstructing his right ventricular outflow tract. Complete surgical excision of the mass with preservation of the pulmonary valve was performed. The diagnosis of myxoma was histologically confirmed.
Conclusion: Cardiac myxomas located in the right ventricular outflow tract are rare and can present unusual diagnostic and therapeutic challenges.