Small bowel lymphomas are rare and constitute approximately 1% of the malignant gastrointestinal tumors. However, the risk of malignant disease in adult celiac disease is about 8-10%, and non-Hodgkin lymphoma is the most common. In the literature, cases with celiac disease and small bowel lymphoma have been reported, but the emphasis on emergency surgery is extremely rare. We herein present a case of primary small intestinal lymphoma diagnosed after surgery in a 55-year-old male patient who presented to our emergency department with findings of gastrointestinal perforation and had a history of celiac disease and dermatitis herpetiformis. The purpose of this report is to review this situation briefly and discuss it in the light of literature.