Objectives: Anaplastic Gangliogliomas (AGG) are very rare tumors of the central nervous system with an incidence of 0.02 cases per million per year. They occur most frequently in the temporal lobe (27%). The surgical resection and the absence of dissemination are important predictors of overall survival.

Material and methods: A 33-year-old male, previously operated to a right amygdala/hippocampal WHO grade I Ganglioglioma in our epilepsy surgery program, presented with severe headaches 6 months after surgery. A brain Magnetic Resonance Imaging (MRI) revealed a local recurrence of the tumor and contrast enhancement in some cranial nerves and cauda equina nerve roots. He was submitted to reopening of the previous right temporal craniotomy. The tumor was approached through the superior temporal sulcus with image-guided surgery with projection of tumor boundaries in the microscope ocular. The surgical video identifying the anatomical landmarks is complemented with pre-operative MRI and post-operative MRI.

Results: The post-operative MRI showed a complete removal of the tumor which was classified as an Anaplastic Ganglioglioma WHO grade III. The patient transiently improved from his symptoms and the postoperative imaging revealed a complete removal of the nodular tumor. He started whole brain radiation therapy and died 6 weeks after surgery from leptomeningeal dissemination.

Conclusions: This video presentation shows the relevant anatomy of the medial temporal region with special emphasis to vascular and cranial nerves relationships and the usefulness of neuronavigation injection into microscope ocular. It also shows an unusual tumor with a very aggressive behavior.