Lipoblastoma is a rare, benign, fatty tissue tumour that occurs in infancy and early childhood. Intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer. In this study, we report the case of a 15-month-old girl who presented with a tumour in the pulmonary valve. A complete resection of the tumour was undertaken, with a histopathology report confirming the diagnosis of lipoblastoma.