Introduction: Cystic angiomatosis of the skull and spine is an exceptionally rare, benign vascular lesion. Both the vertebral bones and the skull may be affected. Diagnosis and treatment of this disease is multidisciplinary.
Discussion: Histological examination is ultimately required to make a diagnosis. When the craniocervical junction is involved, the site of biopsy should be carefully selected so as to reduce procedure-related morbidity, including cerebrospinal fluid leakage and spinal deformity. We present a case report of a 4-year-old boy with cystic angiomatosis of the skull base and upper cervical spine associated with a Chiari I malformation and provide a review of the pertinent literature.