Abstract
BACKGROUND: Lymphedema is the accumulation of a protein-rich fluid in the interstitial space due to reduced lymph transport capacity. Congenital primary lymphedema affecting only one of the upper limbs is a rarity. CASE REPORT: We present a case of an 11-month-old infant presenting with swelling of the right upper limb, which had gradually progressed over the past five months. Lymphoscintigraphy was suggestive of lymphatic blockade in the right upper limb. All other investigations were within normal limits. A diagnosis of primary congenital lymphedema affecting the right upper limb was made. The patient was managed conservatively with complex decongestive therapy and was requested regular follow-up. The lymphedema did not increase within four months of follow-up. CONCLUSIONS: Complex decongestive therapy is the cornerstone of the management of primary congenital lymphedema. New investigational therapies such as leukotriene B4 antagonists hold a promise for patients with lymphedema.