The current mainstay of management of chronic beryllium disease involves cessation of beryllium exposure and use of systemic corticosteroids. However, there are no randomized controlled trials to assess the effect of these interventions on the natural history of this disease. Despite this limitation, it is prudent to remove patients with chronic beryllium disease from further exposure and consider treating progressive disease early with long-term corticosteroids. The effect of treatment should be monitored using pulmonary function tests and high-resolution computed tomography of the chest. However, once pulmonary fibrosis has developed, corticosteroid therapy cannot reverse the damage.