Abstract
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, and liver PEComas are extremely rare. They are usually discovered incidentally, and diagnostic pitfalls are frequent owing to similar imaging characteristics to other liver neoplasms. We present a patient whose evaluation was consistent with a right-sided hepatocellular carcinoma for which right hepatectomy was performed. Based on the final histopathological examination and immunohistochemistry, a diagnosis of PEComa was made. Immunohistochemistry plays a crucial role in arriving at the diagnosis, and resection represents the standard of care. A long-term follow-up is recommended because the natural history of PEComas is unpredictable.