A case of familial cerebral cavernous hemangioma (FCCM) was clinically diagnosed, treated, and genetically analyzed, and relevant literature was reviewed. Methods: The clinical data of the index patient and her relatives were analyzed, and their DNA was sequenced by whole exome sequencing (WES). Results: The index patient and her elder son were clinically diagnosed with FCCM, and the proband had skin vascular malformations. They both carried the c.1307_1308insT heterozygous mutation of the CCM1/KRIT1 gene, which resulted in a frameshift mutation starting from amino acid leucine (Leu) no. 436 and terminating at the sixth amino acid (p.Leu436PhefsTer6). Other asymptomatic family members did not have this mutation. This mutation had not been previously reported. The index patient had bilateral cavernous hemangiomas of the temporal lobe which were difficult to treat. Two surgeries were performed yielding subpar results, and close clinical follow-up was still needed. Conclusion: A new mutation site of CCM1/KRIT1 was discovered in the FCCM patients. In addition to intractable epileptic seizures, the proband presented skin vascular malformations caused by the CCM1 gene mutation. The proband had multiple cavernous hemangioma (CCMs) and suspected epileptogenic foci located in the bilateral temporal lobes; two surgical treatments were performed but were ineffective.