Background Developmental venous anomalies (DVAs) are the most common intracranial vascular malformations of the brain. They are most commonly asymptomatic and often diagnosed incidentally. Illustrative case We present a rare case of a 9-year-old boy with seizures caused by a subcortically located DVA posterior to the postcentral gyrus of the left brain hemisphere. MRI also showed an enlarged, microcystically altered pineal gland and an extracranial cavernous hemangioma. EEG showed left fronto-centro-parietal spikes that corresponded with the location of the DVA. The spikes were occasionally secondary generalized. The boy responded well to oxcarbazepine treatment, and no surgical intervention was required. Control MRI scans showed no progression of the DVA. Conclusion Epilepsy may be caused by DVA. The treatment starts with anticonvulsants. The surgical treatment is the therapeutic choice only if seizures are resistant to conservative therapy because surgery might result in serious ischemic or hemorrhagic complications because DVAs are considered to be variations of normal transmedullary veins that are necessary for the drainage of white and gray matter.