Congenital ranula (CR) is a rare tumor. Few cases of prenatal diagnosis (PD) of CR have been reported in the literature and diagnosis usually is beyond 20 weeks of gestation. We report a case of a pregnant patient with 16 weeks of gestation, where ultrasound (US) visualized in her fetus, a cystic tumor protruding in his oral cavity. Basis and complementary studies, including fetal echocardiography, were normal. Serial two-dimensional (2D) and three-dimensional (3D) US studies monitored fetal growth, amniotic fluid, progressive changes in the cystic mass, and maxillary movements. At 38.5 weeks of gestation, a cesarean section was performed. At 17th neonate days old, the neonate was done the simple and complete excision. Early PD of CR is possible. In this case was done as early as at 16 weeks of gestation. This is the first CR reported case with the earliest PD (16 week of gestation). We recommend that during the first trimester US, the oral cavity must be well explored, to rule out tumors.
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