Background: Renal angiomyolipoma is the most common benign tumor of kidney. It is often associated with pulmonary lymphangioleiomyomatosis specially when it is associated with tuberous sclerosis. This association is often missed and the patient bears the risk of hyperinflation lung injuries during or after the treatment of renal angiomyolipoma. Case Report: We hereby report a patient with pain and swelling in left side of the abdomen suggestive of a renal mass. She underwent left simple nephrectomy which eventually came out to be renal angiomyolipoma. She had other characteristics of tuberous sclerosis for which computed tomography of thorax and guided biopsy was done which revealed the presence of pulmonary lymphangioleiomyomatosis. Conclusion: We should be aware of the co-existence of both renal angiomyolipoma and pulmonary lymphangiomyomatosis specially when other features of tuberous sclerosis are present. We should ensure regular follow-up and treat both the diseases to ensure the complete recovery of the patient.