The most common benign cardiac tumours are the myxomas, the rhabdomyomas, and the fibromas, with the latter 2 variants being the most common tumours encountered in children. The size and location of tumours within the heart create a variety of clinical findings, such as murmurs, chest pain, tachyarrythmias, and congestive cardiac failure. Nowadays, the tumours are usually diagnosed by echocardiography, magnetic resonance imaging and cardiac catheterization. Surgical excision is the treatment of choice if the tumour causes either arrhythmia or cavitary obstruction. In this report, we describe a giant ventricular fibroma co-existing with an atrial septal defect in a girl aged 15 years.