Abdominal angiostrongyliasis (AA) is a disease caused by Angiostrongylus costaricensis, a nematode that can infect humans accidentally through the ingestion of larvae. Worms live inside intestinal small vessels and can lead to gastrointestinal symptoms and bowel necrosis in otherwise healthy patients. Therefore, abdominal angiostrongyliasis may be important in the differential diagnosis with systemic vasculitides and other rheumatic diseases with vascular involvement. We report a case of abdominal angiostrongyliasis in an 18-year-old woman presenting with necrosis of the terminal ileum.