Bronchiolitis obliterans organizing pneumonia (BOOP) is an uncommon pulmonary disorder, the clinical spectrum of which is variable. We present a fatal case of BOOP, which developed spontaneous pneumothorax, a complication considered rare. Unusual was also the upper lobe distribution of the infiltrates. The histologically diagnosed disease failed to respond to antibiotics and corticosteroids and the 74-year-old patient eventually succumbed with acute respiratory distress syndrome, 50 days after disease onset. Spontaneous pneumothorax should be added to the complications of BOOP, which may adversely affect prognosis.