Perivascular epithelioid cell tumor (PEComa) is a multi-potential tumor based on mesenchymal cells distributed around capillaries. The main affected population is female, and the clinical manifestations are not specific. It can affect all parts of the body. There are more PEComa in the uterus and very few PEComa in the liver. Due to its low incidence, clinicians lack awareness of it. Based on the relevant literature, this article reviews the clinicopathological features, imaging features, molecular phenotypes, diagnosis, differential diagnosis, and treatment of liver PEComa, so as to strengthen the understanding of the disease, prevent missed diagnosis and misdiagnosis, and guide clinical work.
