INTRODUCTION: Lymphoma account for only 1 to 4 % of neoplasms in the gastrointestinal tract (GIT), with the stomach being the most common location (68 to 75%) and the small bowel as the second most common (30 %). Among the possible presentations of GIT lymphomas, small bowel perforation is one of the most lethal and rare. Here we present a case of a patient that had a perforated jejunum secondary to lymphoma. CASE DESCRIPTION/METHODS: 57 year old female with history of diabetes, presented with 2 days of severe abdominal pain and vomiting. Physical exam revealed peritoneal signs and absent bowel sounds. CT showed multifocal small bowel thickening, pneumoperitoneum and extraluminal contrast indicating perforation. The patient was rushed to have explorative laparotomy which revealed multiple masses along the small bowel and had resection of a perforated jejunum. Pathology revealed lymphoma. Immunohistochemical staining was positive for CD20 and CD5, and was negative for Bcl-6 and CD10. FISH analysis was negative for translocation t(11,14), t(14,18), and MYC. The patient’s wounds healed and she was eventually treated with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) along with rituximab for 6 cycles and Intrathecal (IT) chemotherapy. DISCUSSION: The WHO classify small bowel lymphomas (SBL) into B-cell subtypes (90 % of cases), T-Cell subtypes and Hodgkins Lymphoma.The most common symptoms are abdominal pain (70% of cases), ileus, and weight loss. Perforation was only found in approximately 16% of cases. CT may show polypoid lesions, multiple masses, and small bowel thickening. Usually, SBL is surgically resected for both diagnosis and treatment. Historically, endoscopy has been difficult because of the location. However, with the advent of capsule endoscopy and double balloon techniques, endoscopy can potentially avoid invasive surgeries. Our patient had an acute abdomen and therefore underwent surgical evaluation. Presence of CD20 and CD5, indicated a CD5 positive B-Cell lymphoma. FISH did not show any genetic rearrangements for Cyclin D- t(11,14), a marker for Mantle cell lymphoma, nor Myc and Bcl, markers for Burkitt’s and Follicular cell lymphomas, leading to the diagnosis of DLBCL. Localized tumors undergo surgical resection followed by radiation. TNM stage IIIE or higher is treated with combination chemotherapy, CHOP and rituximab, as was our patient. SBL is a rare entity; this case provides some insight and we hope such knowledge can only improve the management of patients to come.