Two patients with pneumatosis intestinalis (PI) and mixed connective tissue disease/overlap syndrome are discussed. One patient also presented a retropneumoperitoneum, a feature as yet undescribed in PI. A review of previously reported cases revealed that PI should be suspected in those patients known to have systemic sclerosis-type involvement of the oesophagus and the small bowel, and who present with abdominal distension. PI may occur early in the course of the disease, may resolve rapidly through medical intervention only, and is compatible with a 2-year survival rate.