Cavernous malformation is a relatively rare disease, but is important in the etiology of cerebral hemorrhage in pregnant and puerperal women. The risk of bleeding is particularly high in patients with a previous history of bleeding, patients with a family history of cavernous malformations, and patients with the causative cerebral cavernous malformation gene. Cavernous malformations are more likely to bleed or to increase in size during pregnancy, under the influence of female hormones and vascular growth factors such as vascular endothelial growth factor. We report our strategy for the treatment of cavernous malformations in pregnant women, with reference to the relevant literature. Asymptomatic patients and those with mild symptoms are usually followed up conservatively by magnetic resonance imaging, without active treatment, but surgical treatment is indicated in patients with severe or progressive symptoms; surgery should also be considered in patients with mild symptoms having risk factors for bleeding. If surgical treatment is selected, the operation plan needs to be devised in collaboration with the specialties of anesthesiology and obstetrics and gynecology, rather than by the brain surgeon alone, in view of the possibility of occurrence of complications specific to pregnant women, such as complications related to weight gain and difficulty in securing the airway, which develop during the perioperative period.