Common variable immunodeficiency disease (CVID) is the most common primary immunodeficiency in adults, and multiple organs may be involved. This is a case report of a 49-year-old female patient with granulomatous-lymphocytic interstitial lung disease, liver fibrosis, portal hypertension and rectal cancer. Examinations showed non-necrotic granulomas in her lungs, mediastinal glands and liver, and she was seen in six different specialities. The multifaceted manifestation of CVID calls for multidisciplinary collaboration.