Over a 6-year period (1989-1995), gastrointestinal (GI) perforation was diagnosed in nine preterm infants (mean gestational age 27 weeks, mean birth weight 872 g). Three presented with necrotizing enterocolitis (NEC), two with indwelling-tube-induced perforation of the stomach, one with small-left-colon syndrome, and another with meconium ileus. Spontaneous intestinal perforation occurred in two similar very-low-birth-weight (VLBW) infants, in the distal ileum, on days 8 and 9 of life, respectively. The only clinical sign was extensive abdominal distension, and abdominal X-ray studies revealed free peritoneal air. All findings were distinct from those associated with NEC. Their further clinical course was complicated by reperforation on day 32 and 39, respectively. They subsequently recovered and presented without GI problems at the corrected ages of 4 and 2 months, respectively. In contrast to high mortality of 57% in the group with non-spontaneous intestinal perforations, spontaneous perforation seems to have a good prognosis even in VLBW infants if diagnosed and treated promptly.