We describe a fetal intracerebral hemorrhage associated with familial thrombophilia. Intraventricular and intraparenchymal hemorrhage of the left cerebral hemisphere was diagnosed at 22 weeks of gestation. Neuropathologic examination demonstrated a large germinal zone hemorrhage and ischemic changes secondary to bleeding. The fetus manifested a heterozygous Leiden mutation of the factor V gene, and a heterozygous F76L mutation of the protein C gene. The literature indicates that the coexistence of genetic risk factors of thrombophilia and fetal intracerebral hemorrhage is rare, except in the context of pregnancy-related complications. The utility of laboratory screening is discussed.