Lymphedema is a symptom of tissue fluid accumulation which arises as a consequence of impaired lymphatic drainage. This reduced drainage can be either congenital or acquired, for example after breast cancer treatment. Early diagnosis of a swollen limb and adequate treatment are important in order to prevent irreversible tissue changes. The medical history and characteristic clinical presentation form the cornerstone of the diagnostic process. Lymphoscintigraphy can be used to obtain additional information about the functioning of the lymphatic system. Information and recommendations on precautions, preventive measurements and self-management instructions are important to all patients with (risk of) lymphedema. Treatment for lymphedema has to be adjusted to the patient and may consist of several therapeutic options, including manual lymphatic drainage. After volume reduction has been accomplished, a well-fitted compressive garment is essential in the maintenance phase. Surgical procedures for lymphedema are strictly indicated and should be performed by protocol in a multidisciplinary setting with long-term follow-up. Lymphedema is seen in many medical disciplines. A treatment plan is drawn up on the basis of a thorough knowledge of the diagnostics and treatment, with targeted referral to paramedical personnel. As it is a chronic condition, lymphedema requires life-long treatment and follow-up. In view of the complex nature of lymphedema it is recommended that local lymphedema protocols be developed.