We describe a case of a 65-year-old male with recently diagnosed diffuse cutaneous systemic sclerosis associated with usual interstitial pneumonia and pulmonary hypertension. Patient presented to the emergency department complaining of low-grade fever, increased sputum production, progressive dyspnea and weight loss. High-resolution computed tomography scan showed multifocal bronchiectasis with multiple small nodules. Bronchoalveolar lavage culture was positive for Mycobacterium intracellulare. Antimicrobial treatment was started which improved respiratory symptoms. One month after the initiation of antibiotics, cyclophosphamide therapy was started with adequate tolerance.