Gastrointestinal (GIT) involvement is common in systemic sclerosis and can affect any part of the digestive tract, with significant patient-related loss of quality of life and morbidity. Although dysmotility is the most frequent cause for symptoms, mechanical complications such as colonic volvulus, while rare, can happen during disease course and may be life-threatening. We report the case of a patient who presented to our department with rapidly progressive diffuse cutaneous systemic sclerosis, who developed severe upper and lower GIT dysmotility with significant weight loss. The GIT involvement was complicated by recurrent pseudo-obstruction episodes that resulted in colonic volvulus, which recurred despite surgical and supportive management. Intravenous immunoglobulin (IVIG) was administered at 2g/kg over five consecutive days, with marked clinical and radiological improvement and resolution of obstructive episodes. To our knowledge, this is the first case report that describes the use of IVIG in systemic sclerosis complicated by mechanical colonic volvulus. This case highlights an atypical and aggressive disease phenotype and suggests that IVIG may be a therapeutic option in selected patients with severe and refractory GIT involvement while maintaining a reasonable safety profile.