Systemic sclerosis (SSc) belongs to the family of autoimmune connective tissue diseases and is still a challenge to every practicing physician. The disorder is characterized by progressing fibrosis of the skin and internal organs, abnormal activation of the immune system, and distinct changes in microcirculation. Although it is rare--with a prevalence of about 20:100000--patients need to be cared for in a daily setting. In general thickening of the skin is the first sign of the disease, so dermatologists are most frequently consulted first. Two subtypes exist, limited and diffuse forms. Both entities usually involve internal organs, and therefore interdisciplinary cooperation is mandatory. The increased morbidity and mortality depend predominantly on the grade of involvement of the affected organs. Therefore it is essential to diagnose systemic sclerosis early and to identify and monitor all complications closely. In this respect gastrointestinal involvement is frequently neglected, owing to its primarily non-life-threatening character, resulting in substantially delayed therapy.