A 19-year-old male presented to us with anasarca and a history of chronic diarrhea. Investigations revealed primary intestinal lymphangiectasia leading to protein losing enteropathy. Patient was doing well for 1 year on a modified diet until he got admitted with a severe headache, vomiting, and papilledema. Complete workup demonstrated cerebral venous thrombosis as a result of deficiency of protein S.
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