Lymphangiomatosis is a rare disorder and the underlying aetiology is poorly understood. The diagnosis is difficult, and relies on various clinical, radiological and histological features. Therapy is diverse, with combinations of treatment required to achieve disease control. The prognosis is guarded, and currently a vast amount of work is being undertaken to understand the disease, and to find focused therapy. We present a case of histologically, and radiologically proven lymphangiomatosis in a 4-year-old child who presented with nonspecific symptoms.S Afr Respir J 2017;23(2):35-38.
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