Background: Neutropenic enterocolitis (NEC) also known as typhlitis is an acute, life-threatening inflammation of the small and large bowel often seen in patients with leukaemia undergoing cytotoxic chemotherapy: occasionally this syndrome could present in other immunocompromised patients.
Aim: To report an unusual case of Fanconi anemia complicated by neutropenic enterocolitis.
Case report: We report a case of a 13-year-old boy affected by Fanconi anemia with severe neutropenia and who presented neutropenic enterocolitis. Abdominal ultrasonography showed thickening of cecum and ascending colon of 1.18cm. The outcome was favourable with medical management.
Conclusion: Neutropenic enterocolitis in Fanconi anemia is a rare and a severe illness: however, the outcome is improved with early management.