A 29-year-old man with Crohn's disease involving the ileum and cecum was se
en. He had angioneurotic edema with C1' esterase inhibitor deficiency. Late
r, his 50-year-old mother was evaluated because of abdominal pain. She had
recurrent urticaria, C1' esterase inhibitor deficiency and radiographic stu
dies showed Crohn's disease of the ileum. A maternal family history reveale
d other members affected with either Crohn's disease or angioneurotic edema
. The clinical observations in this family suggest that angioneurotic edema
associated with C1' esterase inhibitor deficiency may be closely linked ge
netically with a familial form of Crohn's disease.