Pial arteriovenous fistula (PAVF) is an extremely rare type of intracranial vascular congenital anomalies. The presented clinical case is a unique example of intrauterine diagnosis of PAVF using fetal MRI at 30 weeks of gestation, which allowed successful surgical treatment in the early neonatal period. The case demonstrates the capabilities of fetal MRI in the diagnosis of PAVF and estimation of accompanying brain changes, which are fully consistent with the results of postnatal cerebral angiography. Based on neuroimaging data, endovascular embolization of the fistula with detachable microcoils was successfully performed on the 2nd day of the child’s life. A good neurologic outcome of the surgery was stated. Taking into account the known unfavorable outcome of PAVF in the case of untimely surgical treatment, this observation demonstrates the need to use fetal MRI for prenatal differential diagnosis of vascular malformations in order to reduce the risk of possible complications and mortality in the early neonatal period.