Background: Lymphangioleiomyomatosis (LAM) is a rare disorder that presents in women of childbearing age. The affected patients present with spontaneous pneumothorax, chylothorax, hemoptysis, and slowly progressive dyspnea. There are poor awareness, knowledge, and records about this disease in Nigeria. Case presentation: We report a case of pulmonary LAM in a 43-year-old woman who presented with progressive shortness of breath with recurrent hemoptysis and pneumothorax. Her imaging and Vascular endothelial growth factor level were in keeping with LAM. The typical features in the current case include the patient's age, gender, radiologic features, and VEGF- D value. Conclusion: There is a need for a high index of suspicion for LAM in women of child-bearing age with cystic lung diseases. There is also a need for registries for rare lung diseases in Africa.