Mesenteric Fibromatosis (MF) is a rare myofibroproliferative tumour that may present as a common clinical entity like intestinal obstruction or abdominal mass. They are exceedingly rare in the pediatric population. They often mimic Gastrointestinal Stroma Tumours (GIST) in their presentation, imaging and even histological features. Here we present the case of a one year, 9 months old boy who had a right hemicolectomy for an obstructing ascending colon tumour whose initial histological analysis was suggestive of GIST. A repeat analysis including Immunohistochemistry staining confirmed the diagnosis of MF. No further treatment was given and the child is asymptomatic one year later. MF though rare may present as common clinical entities and tends to mimic MF. Detailed analysis is required to distinguish it from GIST as the prognosis and treatment may differ significantly.