Background: Primary cardiac sarcomas are the most commonly encountered primary cardiac malignancies with a marked tendency of recurrence. In the complex and heterogeneous field of cardiac masses a proper differential diagnosis based on multimodality imaging approach is extremely useful in order to plan the most appropriate treatment. Case Summary: We report the case of a 69-year-old woman presenting with worsening dyspnoea and syncope due to cardiac pleomorphic sarcoma, emphasizing the critical aspects of differential diagnosis and management. Discussion: A stepwise diagnostic strategy through multimodality imaging evaluation is the cornerstone for an appropriate prompt intervention, even if histopathological characterization remains the diagnostic gold standard for histotype definition, to guide the treatment and define the prognosis.