Abstract Background Solitary fibrous tumor (SFT) are rare spindle cell tumors originating from the mesenchymal cells mostly from the visceral pleura. SFT was first described as a distinct entity in 1931 by Klemperer et al. Until now, we have limited data regarding the manifestation and behavior of extra pleural forms such as cardiac SFT. Here we present a case of SFT involving the pericardium where the diagnosis was made by imaging followed by biopsy findings. We also review the literature of SFT involving the heart and the management approaches. Case Presentation An 81‐year‐old male presented with progressive dyspnea. Computed tomography (CT) of the chest showed a 6.2 × 5.3 cm soft tissue mass in the anterior mediastinum. Further imaging with CT angiogram showed a stalk‐like connection to the pericardium. A biopsy of the mass showed spindle cells positive for BCL‐2, CD34, and STAT 6, indicative of a solitary fibrous tumor. A surveillance approach was adopted for the patient. Conclusion Primary pericardial tumors are exceedingly rare, with a prevalence rate of 0.001%‐0.007%. Diagnosing a SFT requires a positive CD34 and BCL‐2 marker. The current recommendation is resection of localized disease which has been documented to be curative in cases of benign disease however our patient was put on surveillance.
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