Congenital diaphragmatic hernia (CDH) is a known cause of secondary gastric volvulus (GV). Both entities are life-threatening, either alone or in exceedingly rare instances when they occur in combination. Here, we describe one such rare combination of CDH and secondary GV in a nine-year-old boy, who presented to us with recurrent episodes of abdominal pain. Urgent laparotomy was done after radiological evaluation (X-ray of chest and abdomen and contrast-enhanced CT chest and abdomen), which revealed mesenterico-axial volvulus of the stomach, secondary to an underlying diaphragmatic defect in the left hemidiaphragm, thus establishing the cause (diaphragmatic defect) and effect (GV), and resulting in a favourable outcome.