INTRODUCTION: Pulmonary complications of inflammatory bowel disease (IBD) are rare; these include pneumonitis, interstitial lung disease, and pleural effusions. We present a case of eosinophilic pleural effusion and pulmonary hypertension as the presenting extra intestinal manifestations (EIM) in a patient with Crohn’s disease (CD). CASE DESCRIPTION/METHODS: A 63-year-old African American man with recent diagnosis of inflammatory ileocolonic CD treated with a single course of budesonide presented to his pulmonologist with shortness of breath. His CD diagnosis was made on routine screening exam and he was otherwise asymptomatic. CT imaging showed a new right-sided pleural effusion, entrapped lung, and enlarged mediastinal lymph nodes. Echocardiogram revealed pulmonary hypertension (PT). The patient underwent thoracentesis that showed eosinophilic infiltrate without evidence of infection or malignancy. After ten days the patient had recurrence of his pleural effusion, and required repeat thoracentesis. V/Q scan was consistent with chronic thromboembolic pulmonary hypertension (CTEPH). Colonoscopy showed inflammatory CD of the ascending colon and cecum. Lab evaluation showed peripheral eosinophilia, and a positive S. stercoralis serum IgG. He was treated with a course of Ivermectin and started on infliximab infusions with resolution of his effusion and shortness of breath. DISCUSSION: EIM of IBD affect half of all patients. Pulmonary EIM are rare, especially as presenting symptoms for disease. Per our review, this is the first documented case of PH and pleural effusion as the presenting symptoms of CD. We believe the hypercoagulable state induced by CD led to the development of CTEPH. It is unclear what role the S. stercoralis played in this patient’s disease, however, we suspect given distribution and histology the patient had CD and previous S. stercoralis exposure. As his symptoms, effusions, and lymphadenopathy all improved with infliximab, this suggests that the pulmonary manifestations were related to his inflammatory condition. We provide this case as the first description of CTEPH and pleural effusion as an EIM for CD.