Primary cardiac tumors are extremely rare and they most commonly occur in adults. The majority of these neoplasms are benign and, in children, rhabdomyomas, fibromas and teratomas represent the most prevalent subtypes. Among the malignant primary cardiac tumors, sarcomas are the most common. In this case report, we present the first pediatric case of a primary cardiac intimal sarcoma, a histological subtype never previously reported in the pediatric population. Our patient is a 16-year-old who presented with severe cardiogenic shock and arrhythmias and underwent urgent surgical resection of his cardiac mass.