ABSTRACT
A case of lymphomatoid granulomatosis in a previously healthy 13-year-old Thai girl presenting with right sixth cranial nerve palsy and severe upper airway obstruction was reported. Cranial nerve palsy later disappeared spontaneously but the patient developed multiple pulmonary nodules and cavity leading to pulmonary insufficiency. Her course was complicated with septicemia which limited the use of corticosteroid and cytotoxic drugs. She finally expired with pseudomonas sepsis in addition to pulmonary and liver involvement. This is the first case of lymphomatoid granulomatosis in a child ever reported in Thailand. Lymphomatoid granulomatosis should be included in the differential diagnosis of upper airway obstruction with pulmonary nodules and cavity and multi-organ involvement in children.