Object: The authors present their experience with the 17th pediatric intramedullary cavernoma reported in English literature.
Methods: The patient firstly underwent surgery for a left frontal cavernoma when he was 2 years old. Also the child's mother was operated for a C2-C3 intramedullary cavernoma. He grew up normally and the radiological follow-up was negative for other brainstem cavernous malformations. When he was 11 years old he complained a worsening tetraparesis. A cerebral and spinal magnetic resonance (MR) imaging revealed the presence of a C1 intramedullary cavernoma and a pontine cavernoma. He underwent surgery for the cervical lesion, which was completely removed. The postoperative course was regular with a total recovery from the neurological deficit. The boy underwent a radiological follow-up, monitoring the pontine lesion, which spontaneously regressed when he was 19 years old.
Conclusion: The rarity of the pediatric intramedullary cavernoma, the familial occurrence, and the spontaneous regression of the pontine cavernoma make this case very peculiar.