This case reports the successful management of chylothorax in a nontransplanted patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy failed, including total parenteral nutrition, pleural drainage, surgical pleurodesis, and pleurectomy. Commencement of sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of chylothorax after 20 days. Discontinuation of sirolimus for abdominal surgery led to recurrence of the chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years.We conclude that sirolimus should be considered in the management of pLAM-related chylothorax, perhaps before surgical intervention.