<p>Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is defined as a type of lymphoma that occurs in the intestine, but may show extra-intestinal involvement, such as in the skin, brain, lungs, stomach, ovaries, and uterus, which have been previously reported. The disease has no specific clinical manifestations and is often diagnosed by pathological examination as a complication of intestinal perforation or obstruction. The difficulty of making a timely diagnosis is further compounded when the disease begins with non-gastrointestinal symptoms. In this paper, We report a case of MEITL with concurrent abdominal and pulmonary involvement on imaging, but only present with respiratory symptoms. The patient was diagnosed as Peripheral T cell lymphoma, Not otherwise specified (PTCL, <i>NOS</i>) initially based on lung biopsy. However, the diagnosis of MEITL was finally established due to complications of intestinal obstruction and perforation during treatment. Therefore, for lymphomas that occur at multisite outside lymph nodes, multiple-site biopsy should be performed to enhance the accuracy of the pathological diagnosis.</p>