Aim and background: Lymphangiomas are congenital anomalies of the lymphatic system consisting of benign cystic masses of enlarged lymphatic vessels sequestered from the lymphatic circulation and may appear at any site and age. Diagnosis predominantly occurs at birth with 95% located in the cervical, axillary, and head regions. Occasionally, lymphangiomas produce symptoms in neonates by exerting pressure on surrounding tissues, as seen in large masses in the mediastinum and neck regions, as well as infective or hemorrhagic complications. The purpose of this article is to evaluate the clinical presentation and advances in the management of lymphangiomas and to present a case of massive cervical lymphangioma discovered antenatally on the 30th gestational week. Case description: We present a case of a 27-year-old pregnant woman gravida 2, para 1, taking folic acid up to the 9th gestational week. Her brother was born with Down syndrome and died 9 months after birth. On prenatal sonographic examination on the 22nd gestational week, no fetal abnormalities were found. On prenatal ultrasonography at the 30th gestational week, a tumor formation originating from the fetal neck and polyhydramnios were discovered. Following various investigations including prenatal MRI, the diagnosis of massive cervical lymphangioma (cystic hygroma) was made. A male neonate was delivered by cesarean section by the 41st gestational week and is continuously surgically managed by pediatric surgeons and other specialists. Conclusion: Massive lymphangiomas are complicated tumors, which pose management difficulties. They can be effectively managed by staged surgical intervention and multi-professional healthcare teams including surgeons, pediatricians, primary healthcare clinicians, dermatologists. Clinical significance: The case is rare and interesting due to the tumor's massive size and resulting compressive symptoms. Continuous surgical treatment and multidisciplinary management approach were applied, improving the prognosis for the child. This will serve as an education/encouragement for healthcare teams.