Multifocal and skip involvement is quite a rare developmental pattern for primary gastrointestinal lymphomas. A 25-year-old male patient with diffuse large B-cell lymphoma of the small intestine, with macroscopic features and clinical aspects imitating Crohn’s disease and attracting attention with cobblestone-like appearance, is presented herein together with the clinical and pathological features. Multiple ulcerated lesions were also observed infiltrating the serosa with polypoid appearance, 2.5 cm in largest diameter, within the resected jejunoileal specimen, which displayed patchy, healthy-appearing mucosal areas. In microscopic examination, a tumoral infiltration was observed comprised of pleomorphic, atypical lymphoid cells with abundant eosinophilic cytoplasm, marked nucleoli and vesicular nuclei. A B-cell phenotype immunoreaction was observed by vimentin, LCA, CD20, and CD79a in those atypical cells. The diagnosis of the case was diffuse large B-cell lymphoma.The possibility of the presence of this disorder, although rare, is emphasized here for patients applying to the hospital with the signs and symptoms of Crohn’s disease.