Pneumatosisintestinalis (PI) is a radiological finding with about 0.03% incidence and incompletely understood pathogenesis. We report a case of PI with a rare presentation of pneumoperitoneum that underwent diagnostic surgery and finally diagnosed by midgut malrotation and antral stenosis. A 40-year-old man with 1-year history of dyspepsia and vomiting which was aggravated by 20 kg underweight since 3 months ago, despite medications was presented. His imaging examinations showed partial antral obstruction besides pneumoperitoneum and gas collection in the small intestine wall, which were in favour of PI. Samplings of the small intestine during laparotomy reported simple serosal cysts. Persistence of his symptoms forced him to recourse to another hospital and their new imaging revealed Ladds' band in addition to mentioned findings in previous studies; he underwent subtotal gastrectomy plus Ladds' band division by Braun gastrojejunostomy. PI could be a presentation of antral stenosis and midgut malrotation. PI with pneumoperitoneum needs surgical interventions. Considering that most of the midgut malrotation cases are diagnosed in the first year of life and it is very rare in adults, it may bethat these new findings are due to adhesion band formation after the first surgery in this case.