Lymphovascular malformation (LM): formed by abnormal development of lymphatic vessels and can be classified as: microcystic, macrocystic and mixed. Because the lymphatic and venous systems have a common embryonic origin, lymphatic venous malformations can also occur simultaneously. They usually develop after birth or around 2 years of age and are commonly found on the head and neck, and in other areas including the axillae, chest, and perineum. The lesions are soft and compressible. The surface skin is normal and may be pale blue or contain red vesicles. Lymphovascular malformations can lead to local deformities and psychological effects, especially when the lesions are located on the head, face and neck. The most common complications are hemorrhage (35%) and infection (71%). Inadequate control of infection can lead to secondary sepsis. Children with airway involvement may even require tracheotomy. Secondary skeletal overgrowth is another complication, with the mandible being the most common site of involvement, leading to malocclusion and sometimes requiring orthopedic jaw surgery. Lesions in the oral cavity can cause a giant tongue, bleeding, pain, and dental caries. Thoracic and abdominal lesions can lead to celiac disease, celiac pericardium and celiac abdomen. Periorbital lesions can lead to visual disfigurement and blindness. Lymphovascular malformations can be diffuse or multifocal, and even occur in the spleen and develop osteolytic lesions. LM does not require aggressive intervention when the tumor is small and asymptomatic. LM with co-infection requires intravenous antibiotics and generally requires aggressive intervention in the presence of pain, significantly enlarged masses, bleeding, severe deformities and impact on vital organs. Sclerotherapy injection is one method of treating large, symptomatic, large cystic forms of LM. The cystic fluid is first extracted and then injected with drugs that cause scarring of the cystic wall in response to inflammation such as sapropterin, pinyamycin, doxycycline, sodium sulfate (STS), and anhydrous alcohol to obtain a therapeutic effect. Surgical excision is usually feasible in cases where injection therapy is ineffective or where the tumor is multi-housed and cystic, or where the cystic wall remains after injection therapy may be considered for surgical excision. The most common complications of sclerotherapy are skin ulcers, and anhydrous alcohol injections have side effects of systemic toxicity, such as central nervous system depression, pulmonary hypertension, hemolysis, thrombosis, and cardiac arrhythmias. Extravasation of the drug into the muscle can lead to limb atrophy and contracture. It can rebound after injection therapy, requiring long-term follow-up and repeat sclerotherapy, or surgical excision. Some serious complications of LM surgical resection include bleeding, medically induced injury, and local deformity, e.g., 76% of LM resections of the cervicofacial region will have facial nerve injury and 24% will have sublingual nerve injury. Only major resection is usually achieved, with a recurrence rate of 35-64%. For diffuse LM, staged resection and multiple orthopedic procedures can be performed. The comprehensive consideration of treatment options based on the size, type, and location of the lymphangiectasia reflects the degree of the pediatric surgeon’s comprehension, mastery, and skillful application of methods for the disease of lymphangiectasia. We have successfully treated thousands of cases of lymphangioleiomyomata of different types and locations, either alone or in combination with surgical, injectable and pharmacological treatments, with good results and few complications. Recently, the combined thoracic and general surgery treated a case of giant cervical and thoracic giant multiatrial cystic lymphangioleioma referred from other hospitals, and achieved good surgical results.