The perivascular epithelioid cell (PEC) has been proposed to be the proliferating cell type in a group of tumors known as PEComas. The histogenesis of PEComas is one of the most mysterious aspects of pathology. Hypothesis on its precursor are many, including a cell from blood vessel walls or the myoblast. In the current report, we review many morphologic, clinical, ultrastructural, molecular and genetic aspects that support the hypothesis of an origin of PEComas from the neural crest.