Diffuse nodular lymphoid hyperplasia represents a rare disease that is grossly characterized by the presence of numerous visible mucosal nodules measuring up to, and rarely exceeding, 0.5 cm in diameter. These may involve the entire small intestine, the large intestine, or both. The etiology is unknown. When diffuse nodular lymphoid hyperplasia is found predominantly in the colon, it can mimic a variety of polyposis syndromes and this may cause difficulties in diagnosis. The disease may be associated with other pathologies, especially gastrointestinal malignancies. This causes controversy when deciding the treatment options. Following patients without any treatment may lead to malignant progression, while surgical treatment may result in unnecessary radical resections because of obscurity in the diagnosis. We report here a diffuse nodular lymphoid hyperplasia case who underwent a radical resection because of obscurity in the diagnosis.