A 79-year-old woman developed collagenous sprue, a rare small intestinal mucosal disorder. Later, extensive T-cell lymphoma was documented, a neoplasm known to complicate celiac disease. Although the precise relationship of collagenous sprue to celiac disease has been debated and remains controversial, the findings here provide additional evidence that collagenous sprue and celiac disease are closely linked. In the past, long-term survival with collagenous sprue may have been compromised due to severe pan-malabsorption. With improved treatment measures, including modern nutritional support, it is likely that there will be an increased opportunity in future for improved appreciation of the complications of collagenous sprue, specifically, lymphoma.