Intracranial AVMs are typically diagnosed before the patient has reached the age of 40 years, and a few cases have been reported of AVM with skull destruction. We described a rare case of a complex cerebral AVM with skull destruction, presented de novo in 52-year-old woman with epileptic seizures. Neuroimaging investigations revealed complex AVM in right hemisphere as well as extracranially, with signs of skull destructions, likely caused by significant involvement of feeders from external carotid artery. Neurosurgery treatment was not recommended due to morphological characteristics and drainage patterns of the AVM. EEG investigation showed discrete specific activity in correspondent area and pharmacology treatment for seizures was initiated. One year after the initial presentation patient had survived rebleeding episode witch left permanent neurology deficit. This patient considered as a rare case of complex AVM with skull destruction, presented de novo in sixth decade of life.