BACKGROUND There is growing concern about the clinical course of certain diseases in patients who are simultaneously infected by SARS-CoV-2. This report is of a 34-year-old woman from Brazil with a recent diagnosis of pulmonary lymphangioleiomyomatosis (LAM) diagnosed by raised serum VEGF-D levels and the finding of lung cysts on computed tomography (CT) imaging, who presented with COVID-19 pneumonia. CASE REPORT Five months after the diagnosis of pulmonary LAM, which was based on the presence of diffuse and bilateral cystic lesions on CT scan associated with high serum VEGF-D levels, the patient presented with worsening dyspnea, drop in peripheral oxygen oxygenation, fever, and diffuse myalgia. She was using Sirolimus because it inhibits the development of LAM cells. A worsening of lung abnormalities was demonstrated in a chest CT examination, with the appearance of areas of consolidation and ground-glass abnormalities. A nasal swab sample tested positive for SARS-CoV-2 infection using reverse-transcription polymerase chain reaction. Thus, Sirolimus was suspended because of concern about its immunosuppressive action. She received hospital support following the institutional protocol in force at the time, without the need for invasive mechanical ventilation. After 2 weeks, she was discharged from the hospital, with supplemental oxygen at home and return of Sirolimus. CONCLUSIONS This report has described the presentation of COVID-19 pneumonia due to SARS-CoV-2 infection in a 34-year-old woman with a recent diagnosis of LAM involving the lungs.