The extraintestinal manifestations of inflammatory bowel disease have been described in association with almost every organ system. IBD can be associated with several respiratory disorders. Histologically, the nodules usually show sterile aggregates of neutrophils containing areas of necrosis. We found published reports of 15 cases of pulmonary nodules associated with IBD, as of august 2011, predominantly as a manifestation of Crohn’s disease (CD) A 17- year-old male patient was diagnosed with ulcerative colitis (UC), with extensive colitis, and primary sclerosing cholangitis (PSC) 2 years ago. He had been treated with topical combined with oral mesalazine and ursodeoxycholic acid since the diagnosis of the disease. The patient started on azathioprine 3 months ago. He was current in remission, but increased ESR and CRP still remained. He presented with symptoms of cough, pleuritic chest pain and mild dyspnea. He described no fevers. He was treated with ceftriaxone and azithromycin, azathioprine was stopped, but it appeared to be ineffective. CT of the thorax revealed pleural effusion and multiple small pulmonary nodules in both lungs. Open lung biopsy showed a granuloma with central coagulative and lytic necrosis, neutrophils in the periphery and foamy histiocytes, consistent with necrobiotic nodules, interstitial pneumonia, organizing pneumonia and focal bronchiolitis obliterans. His respiratory symptoms improved with a course of prednisone (1 mg/kg/day). A follow-up CT scan showed resolution of the pleural effusion and a >75% reduction in pulmonary nodules size. Respiratory manifestations can be observed in patients with IBD, but they remain uncommon. The incidence of respiratory involvement is greater in UC than in CD, although the necrobiotic nodules have been reported most frequently in CD. We report a case of UC associated with PSC presenting lung involvement with predominance of necrobiotic nodules and serositis. Unlike other cases, this patient presents at younger age than usual. Of 15 reported patients with the association of IBD and necrobiotic nodules, CD was present in 10 patients and UC in five (aged 20 to 75 years). The respiratory disease can develop at any point during the course of IBD. Most patients have respiratory and constitutional symptoms, but quiescent bowel disease, as occurred in this case. Most cases responded well to steroids, but the exclusion of an infectious cause is mandatory. In most reported cases steroid therapy was prescribed alone, however in some patients there was an association with other drugs such as immunosuppressive drugs and infliximab. Clinical and radiologic spontaneous resolution, although controversial, has been reported, which may raise questions about the optimal therapeutic management for this condition. Pulmonary necrobiotic nodules are a rare manifestation of IBD. Although steroid therapy provides favorable clinical and radiological response, further studies are needed to define the best approach.