The connective tissue diseases (CTD) are frequently associated with Interestitial lung diseases (ILD). The presence of an ILD in a patient with CTD is associated with a higher morbidity and mortality. Management of a patient with connective tissue interstitial lung disease (CTD-ILD) depends on disease severity with a focus on improving quality of life. Therapeutic interventions include medications, which aim to prevent progression to fibrosis and decrease inflammation or alveolitis, supplemental oxygen; and pulmonary rehabilitation. Comorbidities including gastroesophageal reflux disease (GERD) and obstructive sleep apnea should be addressed in all CTD-ILD patients. Appropriate referral to support groups, lung transplantation centers, and palliative care are considerations for all patients with CTDILD. The management of a patient with CTD-ILD is a multidisciplinary personalized process that is driven by the specific disease, clinical pattern, and associated comorbidities.