Kaposi sarcoma (KS) is the most commonly diagnosed malignancy in HIV-infected patients. With new treatments, incidence and severity of KS have significantly decreased. A 57-year-old African American male with medical history of AIDS presented with progressively worsening cough, shortness of breath, fever, night sweats, and 60 lb weight loss. On physical examination, he had diffused dark purple skin lesions and decreased air entry in the right lower lung fields. Chest x-ray and subsequent chest computed tomography (CT) showed moderate right lung pleural effusion with scattered bilateral diffuse infiltrates. The patient's absolute CD4 count was 27 cells/microliter. Thoracentesis was negative for infection or malignancy. He was started on chemotherapy paclitaxel along with HAART for extensive pulmonary KS. Since starting the treatment, his condition has significantly improved with near complete resolution of the pleural effusion, oral, and skin lesions. In conclusion, the diagnosis of AIDS-related pulmonary KS is often clinical, typically based on the presence of mucocutaneous disease and compatible features on CT chest. The differential diagnosis of pulmonary KS is broad. A detailed evaluation should exclude an infectious etiology or other tumors. Chemotherapy along with HAART can be used for treatment of severe pulmonary KS.