Intestinal malrotation is estimated to occur in approximately 1 in 500 live births, although symptomatic cases are considerably less common. Midgut volvulus is a potentially devastating complication of malrotation that requires urgent evaluation because it may rapidly progress to intestinal ischemia and bowel loss. However, plain radiographs may be misleading when proximal obstruction produces a double-bubble-like appearance. We report a term female neonate who developed bilious vomiting on the first postnatal day. Initial radiography suggested duodenal atresia. Clinical deterioration with leukocytosis, elevated C-reactive protein, and rising lactate prompted urgent laparotomy at 48 hours of life. Severe midgut volvulus with three complete turns and near-total small-bowel ischemia was identified. Detorsion and planned second-look laparotomy allowed bowel preservation and completion of the Ladd procedure. Persistent postoperative obstruction later revealed a 30-cm post-ischemic fibrotic stricture requiring resection. Approximately 80 cm of small bowel and the colon were preserved. Midgut volvulus should remain in the differential diagnosis of neonatal bilious vomiting, even when radiography suggests duodenal atresia.