Severe intestinal hypomotility in scleroderma is an uncommon but difficult management problem. We present a case of advanced scleroderma bowel complicated by colonic pseudo-obstruction, which was dramatically improved by subtotal colectomy. Benign pneumoperitoneum due to pneumatosis cystoides intestinales later developed. Symptoms and the pneumoperitoneum resolved with oxygen therapy and total parenteral nutrition, followed by an elemental diet. Our case illustrates how a judicious combination of medical and surgical therapy can be successful in advanced scleroderma bowel. The pathology and pathogenesis of intestinal scleroderma are reviewed to provide an understanding of these complications and a rationale for management.