Cerebral cavernous malformations (CCMs) are vascular lesions characterized by enlarged and irregular structure of small blood vessels in the brain, which can result in increased risk of stroke, focal neurological defects and seizures. CCMs can occur as a sporadic or familial autosomal dominant form. Three different genes, CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10, have now been identified as the main targets which are involved in the CCMs’ progression. These three CCM proteins have similar or unique function in maintaining the normal structure of small blood vessels. However, CCM3 mutation results in a more severe form of the disease which may suggest we should pay more attention on the area of CCM3. The current research focused on the angiogenic function and mechanisms of CCM3 including endothelial cell junction, proliferation, migration and permeability, and these findings may offer some potential targets for CCMs’ therapy.