Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus

•PECOMA is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers showing a variable course.•Association of SLE with hepatic PECOMA is unknown.•Surgical resection is the preferred therapy. Perivascular epithelioid cell tumor (PECOMA) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers showing a benign course,although malignant tumors have also been reported. To date there are approximately 33 cases of published hepatic pecomas. We describe a 47-year-old man with a 27-year past medical history of systemic lupus erythematosus (SLE) who underwent left liver lobectomy due to a liver pecoma. His postoperative course complicated with infection, thrombosis of hepatic artery and liver ischemia as well as drug fever. Treatment protocol especially for hepatic PECOMA has not reached a consensus although surgical resection is the preferred therapy. This is the first case of coexistence of liver pecoma and SLE.