Background <p>Non-Hodgkin lymphoma is one of the most common types of lymphoma affecting the gastrointestinal tract. This malignancy can originate from either B-lymphocytes or T-lymphocytes. Intestinal perforation due to non-Hodgkin lymphoma is a relatively rare occurrence, and when it does happen, it typically arises after the initiation of chemotherapy. In the reported case, the perforation happened prior to the chemotherapy, making the case unusual. We aim to highlight the importance of maintaining a high index of suspicion and the critical role of timely, multidisciplinary management in improving patient outcomes.</p> Clinical presentation <p>A 27-year-old male farmer from a rural area in North West Gondar, Ethiopia, was referred to our hospital for further evaluation and management. He initially presented to the referring facility with a 1-week history of abdominal pain. The pain began as a periumbilical discomfort during the first 5 days and later progressed to become diffuse, involving all regions of the abdomen. He also reported three episodes of vomiting and a high-grade fever that had persisted for the last 2 days. Due to lack of timely histopathologic evaluation, confirmatory diagnosis was done after 1 month of surgical intervention.</p> Conclusion <p>Intestinal perforation caused by lymphoma is a rare occurrence in clinical practice, especially before the initiation of chemotherapy. When such perforation does occur, it is often misdiagnosed or diagnosed late due to its atypical clinical presentation. In many cases, the diagnosis is only established postoperatively following histopathological examination.</p>