DISCUSSION: TSC is characterised by the presence of multiorgan hamartomas related to TSC1 or TSC2 gene mutations. Hamartomas in TSC are more frequent in the skin, brain, kidneys and can occur in the lung, heart, small blood vessels, but very seldom in large and medium sized vessels. PAA with TCS is exceptionally rare and has only been reported in 5 cases. All except one case were solitary aneurysms. We report here a unique case of TSC presenting with gigantic PA (main PA 7.8cm, left PA- 6.2cm) along with multiple PAAs, compressing right PV/RA and presented as respiratory failure. To our knowledge, there has not been any reported case of PV/LA compression caused by PAA.