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Lipoblastoma is a rare, benign, fatty tissue tumour tumor that occurs in infancy and early childhood. Intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer. To report the first case of a lipoblastoma in the pulmonary valve. In this study, we report the case of a 15-month-old girl who presented with a tumour in the pulmonary valve. The tumour was completely resected macroscopically. Histological examination of the lesion confirmed the diagnosis of lipoblastoma. Most primary cardiac tu