A pial arteriovenous fistula (PAVF) is a rare neurovascular pathology consisting of a direct connection between one or more arterial feeders and a single venous channel. A PAVF was previously considered a subtype of an arteriovenous malformation (AVM). In the current literature these are distinguished as two independent entities with different clinical, physiopathologic, and structural characteristics. We report a case of a surgically treated 37-year-old woman with an extremely rare combination of an unruptured PAVF and AVM.