Gastrointestinal (GI) involvement is not rarely encountered in Churg-Strauss syndrome (CSS). We describe the case of a young white woman presenting with acute acalculous cholecystitis, who subsequently developed, despite prompt administration of immunosuppressive therapy, life threatening GI involvement, requiring multiple operations. Over a 7-month period the patient eventually recovered from her disease. Forty-two months later she is free from symptoms, with low daily doses (6 mg) of oral methylprednisolone. Although medical and surgical complications of CSS may develop while the patient is undergoing therapy, early diagnosis with joint surgical and medical management is essential to bring the disease under control.