Calcifying fibrous tumour is a rare benign fibrous lesion. It is paucicellular, with fibroblasts, dense collagenisation, psammomatous and dystrophic calcification, and patchy lymphoplasmacytic infiltrates. Calcifying fibrous tumour was first described in subcutaneous and deep soft tissues, and has been reported all over the body. However, calcifying fibrous tumour originating from the heart is extremely rare. This article describes the case of a giant calcifying fibrous tumour arising from the right ventricle in a child, where the tumour was totally resected and no recurrence was observed during a 4-year follow-up period.