Cardiac lipoma, which are primary cardiac tumors, are rare entities often detected incidentally during imaging. There have been very few reports on the right ventricle (RV) lipoma. Here, we present a case of RV infiltrating lipoma involving the interventricular septum (IVS) and the tricuspid valve. Clinical symptoms, diagnostic procedures, multimodality imaging characteristics, and treatment are discussed, and the complete clinical data of this case and relevant details of retrospective literature are reviewed. The study described the case of a 48-year-old woman who suffered from occasional palpitation after exertion for 10 years. Imaging examinations, including echocardiography and cardiovascular magnetic resonance imaging (MRI), revealed a large mass adherent to the IVS and the right ventricular wall that was consistent with lipoma. The patient underwent surgical repair of the tricuspid valve and excision of the partial mass. The gross specimen revealed piles of 5 × 4 × 3 cm fragments with yellowish appearance and pathological results showed infiltrating lipoma.Lipoma is often asymptomatic and diagnosed incidentally. Surgical excision is the main therapeutic intervention, which is always performed in cases of symptomatic lipoma or when malignancy is suspected. Multimodality imaging would be great help in the diagnosis of cardiac lipoma. Echocardiography is a convenient method for follow-up.