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Primary tumor of falciform ligament is exceedingly uncommon. So far, seven cases of Perivascular Epithelioid Cell tumor (PECom), a recently described histological entity with characteristic perivascular tumor cell arrangement and expression of muscle and melanocytic marker by the cells, have been reported in literature. Here, we report a case of PEComa of falciform ligament, which presented with a huge abdominal lump. Pre-surgical CT scan of abdomen was uninformative regarding the nature and organ of origin of the tumor. On laparotomy, it was surprisingly found to arise from the falciform ligament, and this was easily removed. Histopathological diagnosis confirmed PEComa. There was no recurrence of disease after two years of follow up.

Perivascular epithelioid cell tumor (PEComa) of falciform ligament — an extremely rare tumor