ABSTRACT: In this case, we reported a 9-year-old girl who presented with a superior mesenteric venous thrombosis secondary to Protein C deficiency. This condition of venous thrombosis secondary to Protein C deficiency is rare. Symptoms are not specific and may include vomiting, nausea, and abdominal pain. The article describes the case of a familial disorder. Treatment included anticoagulation to prevent further thrombotic events and a resection of the necrotic bowel segment.