A 39-year-old African American woman with sarcoidosis developed chylous ascites. Computed tomography (CT) scan showed extensive thoracic, mesenteric, and retroperitoneal lymphadenopathy. Initial therapy with corticosteroids was unsuccessful in managing ascites and she needed frequent large-volume paracentesis for symptomatic relief. Methotrexate was effective in treating the condition and the patient has remained symptom-free for > 2 years. Chylous ascites secondary to sarcoidosis is extremely rare and this is the first reported case of successful treatment with methotrexate.