Congenital Diaphragmatic Hernia (CDH) is commonly diagnosed in antenatal scans or presents in the neonatal age group. Presentation of CDH in infants or older children with respiratory symptoms is less common. Similarly, the presentation of CDH with primarily gastrointestinal symptoms is also quite rare and poses a diagnostic and therapeutic challenge. An atypical case of CDH with later presentation with gastrointestinal symptoms is described. A failure to diagnose and correct it in a timely fashion could have a deleterious impact on the outcome. A case of a five-month-old child presenting with features of bowel obstruction is described. On evaluation, the child had rapidly progressive respiratory distress and gross abdominal distension, which was found to be due to left-sided CDH. A closed-loop obstruction with impending strangulation was managed with prompt surgery. The patient had a favorable outcome.Late presenting CDH may present with primarily gastrointestinal symptoms with impending respiratory compromise. A high index of suspicion is necessary to ensure early diagnosis and management.