Objective: A description of the clinical presentation and management of a patient with granulocytic sarcoma of the small bowel. Design: Retrospective. Setting: Tertiary. Patients, participants: One patient. Interventions: Laparotomy, biopsy and induction chemotherapy. Main outcome measure: Resolution of computed tomographic (CT) images of intra-abdominal disease. Results: Thirty months disease-free survival. Conclusions: Granulocytic sarcoma can be very chemosensitive and the diagnosis should be considered in patients with small bowel neoplasms and when the operative appearances suggest widespread intra-abdominal neoplastic disease.