Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. She had other features like arthritis, Raynaud’s phenomena, dyspnoea, heaviness of chest, and dysphagia. On investigation, she was strongly positive for antinuclear antibody and Scl 70. Imaging revealed interstitial lung disease (nonspecific interstitial pneumonia pattern) and a fusiform dilatation of ascending aorta of 6.5 cm. Patient was offered surgical correction for aneurysm, for which she refused. To the best of our knowledge, our case report adds up to the few cases of ascending aorta aneurysm in scleroderma available in world literature.

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