Subcutaneous mixed venous-lymphatic malformations are usually seen as proliferative tumors such as hemangiomas and/or lymphangioma [1]. Isolated macroglossia is rarely detected prenatally, and even when associated with Beckwith-Wiedemann syndrome, the diagnosis is typically made after birth [4]. In the case reported here, accurate prenatal diagnosis, by means of ultrasound and fetal MRI, of a subcutaneous mixed venous-lymphatic malformation associated with macroglossia aided the genetic counseling and the parents’ decision-making process and allowed appropriate antenatal and post-natal care management [5]. In addition, adding fetal MRI to ultrasound and Doppler study of the fetal vascular anomalies enhances the characterization of these lesions and the accuracy of the antenatal diagnosis and assists with patient selection for an EXIT procedure [6, 7].