We report an immunodeficient patient with a rare gastrointestinal manifestation. A 26-year-old male with common variable immunodeficiency (CVID) and bronchiolitis obliterans, who was on intravenous gamma-globulin and prednisone, presented diffuse abdominal pain, nausea, vomiting and constipation of 3 days' duration. He reported 5 years of recurrent respiratory infections and diarrhea with negative stool tests, including tests for Strongyloides stercoralis. A physical exam revealed a poor general condition, anemia, dehydration and a distended painful abdomen with guarding, without abdominal sounds. The radiological study showed marked dilation of the small bowel that was edematous. Resection of the affected loop was performed and the histopathologic study showed transmural infection with S. stercoralis and hemorrhagic necrosis of the muscular layer, without mucosal destruction. The patient developed malabsorption syndrome and septic shock; he was treated with antibiotics and thiabendazole and was finally discharged in a good general condition. CVID is a rare disease and its association with systemic strongyloidiasis is very uncommon, but it has been reported in patients on corticosteroids. Hemorrhagic necrosis of the muscular layer without mucosal destruction was not found in the literature studied.