Lymphangioleiomyomatosis (LAM) is a multisystem disorder predominantly affecting women, characterized by cystic lung lesions, abdominal angiomyolipomas (AMLs), and lymphatic abnormalities. It may be sporadic or may also occur in tuberous sclerosis (TSC), an AD disorder resulting from germline mutations in TSC1 and TSC2 gene. Lymphangioleiomyomatosis occurs in 30% of cases with the condition of TSC. Patients with TSC-LAM have the features of TSC in the form of hamartomas, developmental delay, and cutaneous manifestations, such as ash-leaf macules, facial angiofibromas, Shagreen patches, and seizures. Breathlessness being the most common symptom followed by spontaneous pneumothorax. Pneumothorax occurs in 60–70% of the LAM patients with the recurrence rate of 70%, which is the highest among all chronic lung diseases. A 22-year-old female presented with sudden onset of dyspnea and pleuritic chest pain. O/E red papules on face involving nose, nasolabial folds, cheek, hypopigmented macules on legs are seen. The past H/O recurrent pneumothorax with repeated tube thoracostomy revealed the presence for 6 times in the past 2 years. H/O seizures revealed the occurrence of seizures from the childhood. Absent breath sounds on left side was observed on auscultation. Further investigations were done. Her complete blood count was normal. Chest radiograph showed multiple cystic lucencies present in the right upper and middle lobes. Left-sided pneumothorax was present. Computed tomography of the chest showed multiple large cysts in the bilateral upper lobes and small cystic lesions in bilateral lower lobes. Pneumothorax was present on left side. Multiple sub-pleural exophytic cysts were seen at the apex. Computed tomography of abdomen showed multiple variable-sized hepatic lipomatous tumors likely AML. Both kidneys are enlarged showing variable-sized cysts and small AMLs. The MRI of brain had multiple bilateral T2 hypodense signal intensity in the bilateral periventricular region. Bilateral patchy lesions in the subcortical regions were present. Then she was managed with ICD tube thoracostomy, pleurodesis, tab. sirolimus. Conclusion: Definite LAM may be diagnosed in the presence of characteristic lung high-resolution computed tomography (HRCT) and any of the following: angiomyolipoma (kidney)/thoracic or abdominal chylous effusion/lymphangioleiomyoma or lymphadenopathy/TSC. Lymphangioleiomyomatosis is a disease of women that usually occurs at the reproductive age. Lymphangioleiomyomatosis should be in differential diagnosis of cystic lung disease and recurrent pneumothorax.