Intramedullary spinal cavernoma is a rare vascular disease constituting 5-12% of all spinal vascular tumors. The clinical course is usually characterized either by an acute neurological deterioration, recurrent episodes of neurological deficits or by a slowly progressive neurological decline. Microsurgical removal is recommended when the symptoms become clinically relevant and the lesion appears accessible. In this article, we present a surgical technique to completely resect an intramedullary cavernoma with the aid of intraoperative electrophysiological monitoring and intraoperative real-time ultrasound guidance. A brief description of current management of this pathology is also presented.