Organizing pneumonia is characterized by a distinct histologic pattern in the lung interstitium and presents clinically as hypoxemia, fever, cough, and dyspnea that is not attributable to concurrent infection. Typical etiologies of this condition include inflammatory disease, malignancy, toxic inhalation, and an array of medications including the mTOR inhibitor everolimus. In this report, we describe the case of a female with tuberous sclerosis complex on everolimus therapy for renal angiomyolipomas who presented to the hospital with persistent cough, dyspnea, and fevers and bilateral lower lobe opacities on chest X-ray despite multiple courses of antibiotic therapy. Bronchoscopy was performed with transbronchial biopsies, and results demonstrated a lymphocytic predominance and pathologic findings of intraluminal plugs composed of fibroblasts and myofibroblasts consistent with organizing pneumonia. Everolimus therapy was discontinued and patient completed a steroid course with resolution of symptoms. To our knowledge, this is the first published case of organizing pneumonia secondary to everolimus in a patient with tuberous sclerosis complex.