ABSTRACT
Lymphedema, lymphatic fluid is due to an abnormal accumulation in the body in a regional body edema. Congenital lymphedema represents all forms that are clinically evident at birth and accounts for 10-25% of all primary lymphedema cases. The patient was born from consanguineous parents as the first child after two abortion and history of a child death at 14 month of age. On examination the patient had pitting edema at all extremities and abdomen. Lymphangiosintigraphy was performed and no lymph nodes were seen so hereditary primary lymphedema diagnosis was confirmed. Primarily, often caused by a congenital anomaly or dysfunction. As a secondary, acquired disorder is caused by lymphatic flow. Congenital lymphedema, occurs in the first weeks of life. After resolution of the edema the patient was malnourished and had feeding problems. In English literature, congenital lymphedema case with organomegaly, ascites and pitting generalized edema is rarely defined. We report this case because of its unique presentation