Anterior cranial fossa (ACF) dural arteriovenous fistulas (ACF DAVFs) are an infrequent subtype of cranial DAVFs that are usually fed by the anterior ethmoidal artery (AEA) and ophthalmic artery (OA) branches. Due to the lack of dural venous sinus in the ACF, they directly drain into cortical veins, resulting in high bleeding tendency. For this reason, ACF DAVFs have to be treated regardless of whether they are symptomatic or not. A 74-year-old man, with hypertension history, came to our attention because of ear pain, dizziness, and impaired hearing for 2 months. No other pathological conditions have been found in his medical history. The patient underwent brain magnetic resonance imaging (MRI) scan and subsequently second level diagnostic investigations with digital subtraction angiography (DSA), showing a foramen caecum (FC) patency and a persistent, enlarged, arterialized FC vein (FCV) involved in an incidental ACF DAVF (Cognard IV), fed mainly by sphenopalatine arteries (SPAs) branches bilaterally. The patient underwent open surgery performed by small high frontal craniotomy with DAVFs complete closure and without any complications. To the best of our knowledge, this is the first case ever described of FCV persistence with involvement in an intracranial vascular malformation. It has been managed by surgical intervention that can be considered, despite the large skin incision, a minimally invasive approach with an excellent cosmetic result and minimal risk of complications.