Objective To discuss the clinical characteristics， diagnosis processes， and treatment methods of one patient with congenital intrabdominal hernia，and to summarize the potential misconceptions during the diagnostic and treatment processes， and to improve the clinicians’ awareness of this disease. Methods The clinical data and auxiliary examination results of one patient with congenital intrabdominal hernia were collected and analyzed， and the related literatures were reviewed. Results The patient， a 65-year-old male， sought care at the local hospital due to upper abdominal pain before 2 d；there were no significant abnormalities in the examination results at the cocal hospital；blood glucose>25 mmol·L^－1.After receiving hypoglycemic， rehydration， and blood purification treatment， the condition of the patient was worsened， presenting with confusion， hypotension， and respiratory distress；the patient admitted in our hospital for further diagnosis and treatment.After admission，the patient was given despite fluid resuscitation， mechanical ventilation， and supportive treatment， but there was no improvement in the symptoms；interventional radiology was performed angiography of the abdominal artery and right femoral vein， which showed no significant vascular abnormalities in the abdomen. An abdominal paracentesis yielded a mixed bloody fluid， suggesting the concealed intraperitoneal disease； exploratory laparotomy was performed. During operation， the intrabdominal hernia with small intestine necrosis and septic shock were diagnosed， and partial small intestine resection， anastomosis， adhesiolysis， and abdominal irrigation and drainage were carried out. The patient had a good recovery and was discharged on the 14th day after operation. Conclusion Congenital intrabdominal hernia is a very rare cause of intestinal obstruction in the adults，and high suspicion for intrabdominal hernia is one of the differential diagnosis for atypical acute abdomen；early multidisciplinary intervention can be lifesaving for the patients.