Lymphangioleioma is a benign tumor formed by the developmental malformation of lymphatic vessels. It is divided into three types according to its clinical manifestations and tissue characteristics: capillary, spongy and cystic. Lymphangioleioma is often congenital, mostly seen in infants and adolescents, and is found in the skin, subcutaneous and mucous membranes, most commonly in the tongue, lips, cheeks, oral mucosa and the neck and submandibular area. Capillary lymphangioleioma often forms more corn-like white or translucent vesicles on the surface of soft tissues (tongue, lips, and cheek mucosa), which are soft and non-compressible. When combined with capillary hemangioma, it is called vascular tumor. Spongiform lymphangioleiomas are found in the subcutaneous tissue of the cheek and can affect the whole skin. Cystic lymphoma, also known as cystic hydatid tumor, occurs in infants and children, often at birth, and is usually located in the submandibular and posterior cervical triangle. It is soft and fluctuating, with a yellowish, clear fluid extracted by puncture, and is negative on compression and postural tests. Treatment of lymphangioleioma: 1.Sclerotherapy and intracavitary injection of pinyamycin can be applied to all types of lymphangioleioma, especially the recent use of intracavitary injection of pinyamycin has achieved satisfactory results in the treatment of cystic lymphangioleioma in infants and children, which greatly reduces the damage caused by surgery to infants and children’s bodies. 2.Surgery: Surgery is suitable for all types of lymphangioleioma and is the most important treatment means. However, since lymphangiectasia is a benign tumor, lesions with large scope should be partially removed to avoid deformation of normal tissues. For residual tumors, other treatment methods such as second-stage surgery, sclerotherapy and pinyamycin injection can be considered.