HighlightsSystemic sclerosis is a very rare progressive multisystem autoimmune disorder affecting multiple internal organs.Presents a challenge to the physician, anesthetist and operating surgeon involved in medical care of these patients.A multidisciplinary approach should be followed with all of these patients to avoid catastrophic results.We intend to highlight the medical, anesthetic and surgical challenges faced by our team in the management process.We combined the case report with detail review of this rare disease from text books and recent literature.