Background and Clinical Significance: Extremely premature infants face complex medical challenges requiring comprehensive multidisciplinary care. Gastrointestinal malformations, while rare, pose significant diagnostic and therapeutic challenges in this vulnerable population. Case Presentation: We report a case of an extremely premature infant born at 26 weeks gestation with very low birth weight (950 g) who developed a digestive pathology rarely encountered in neonatal intensive care: microcolon, which required surgical consultation and intervention, followed by an atypical postoperative course. Conclusions: The early recognition of gastrointestinal malformations in extremely premature infants requires high clinical suspicion and prompt multidisciplinary intervention. Despite complex postoperative course, favorable outcomes are achievable with coordinated care.