A massive cervicothoracal lymphangioma was diagnosed in a fetus at 25 weeks of gestation. On ultrasound study, the mass showed septated, cystic components and extended from the right submandibular region to the right anterolateral thoracic wall including the right axilla and right scapula. Close sonographic follow-up revealed an increase in the size of the lymphangioma without fetal hydrops. An interdisciplinary approach including a pediatric surgeon, neonatologist, perinatologist and anesthesiologist was chosen. Elective cesarean section under general anesthesia was planned at 37 + 0 weeks of gestation. Surgical correction of the lymphangioma was successfully performed on the 4th day of life. Possible differential diagnoses and the obstetrical management are presented.
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