ABSTRACT
A 40-year-old Caucasian female patient presented to the outpatient General Surgery ward in "V. I. Lenin" Teaching Hospital complaining of a recurrent mesogastric pain that had lasted for 3 months. Physical examination showed a palpable mass confined to that area. She was then admitted with diagnosis of an abdominal tumour. Diagnostic work-up revealed that the process involved the round ligament of the liver, which is an exceptional localization, which motivated us to publish this case after surgical treatment by excision, having also taken into account the results of histopathology which revealed a PEComa, confirmed by inmunohistochemistry. After reviewing the available literature, the low incidence of these lesions, as well as the unusual histological variety, makes the present case one of interest.