Highlights. The clinical case report describes the important role of MRI in the perinatal diagnosis of dural sinus thrombosis, timely verification of the diagnosis, differential diagnosis with other vascular and tumor formations of the brain.Abstract. Dural sinus thrombosis (DST) is a rare congenital anomaly. Ultrasound findings usually mimic intracranial tumors. As a result, the condition is often misdiagnosed and its frequency in general population is underestimated. According to literature data, the progression of DST is a favorable prognosis in case of absence of other concomitant pathologies of the fetal brain. We present a case of DST that was detected with ultrasound, and verified by numerous pre and postnatal MRI starting from 22 weeks of gestation till 4 years of life. A case with a favorable neurological outcome, postnatal ventriculosubgaleal shunting, and ventriculoperitoneal shunting was followed with MRI and CT before and after interventions. The aspects of differential diagnosis, factors determining prognosis are presented. The presented case demonstrates a significant role of fetal MRI in DST diagnosis, depiction of the internal structure of the expanded sinus, and exclusion of concomitant pathology of the brain. These MRI data are integral in determining the appropriate perinatal management, and can be used as predictors of the favorable neurological outcome despite of the severity and size of prenatally diagnosed DSТ.