Intrauterine midgut volvulus is a rare and potentially life-threatening congenital disease that can lead to intestinal ischemia, sepsis and peritonitis caused by bowel perforation and meconium obstruction. Early detection and immediate treatment is crucial to improve the outcome. Herein, we report a preterm infant of 30 weeks of gestation with intrauterine midgut volvulus associated with meconium peritonitis who survived after cesarean delivery and immediate postnatal surgical intervention. The outcome of in-utero intestinal volvulus depends on optimal delivery timing and adequate postnatal treatment. Therefore, prompt multidisciplinary consultation and planning with obstetricians, neonatologists and pediatric surgeons is necessary to reduce the morbidity and mortality associated with fetal midgut volvulus, especially in the preterm period.