A patient with antinuclear antibody (ANA) negative systemic lupus erythematosus (SLE) developed pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum. The literature of pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum is reviewed, the benignancy of these conditions is emphasized, and particular attention directed to their association with and implications in the collagen vascular diseases. This is the first reported case of pneumatosis cystoides intestinalis and pneumoperitoneum in a patient with ANA negative SLE.