A 7-year-old boy presented with adrenal insufficiency. He subsequently developed venous thrombosis in the limbs and was diagnosed with primary antiphospholipid syndrome (PAPS) based on clinical and laboratory parameters. Both adrenals were normal on imaging. He required thrombolysis and anticoagulation. The progressive course of PAPS was controlled with methylprednisolone. There are few reports of PAPS in pediatric patients, and associated adrenal involvement is rare. The unusual presentation, course and management of the patient and of four other reported children with adrenal insufficiency heralding manifestation of PAPS are discussed.
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