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Diaphragmatic hernia is an unusual finding, especially in emergency settings and in the absence of trauma. Imaging plays a crucial role, with various CT signs of diaphragmatic rupture having been described, including the “dangling diaphragm,” “absent diaphragm,” “collar sign,” “hump sign,” “fascia sign,” and “dependent viscera sign”. We report an unusual case of a 53-year-old woman who presented with exertional dyspnea and asthenia. A CT scan was performed, revealing a large right diaphragmatic hernia involving an abnormally rotated liver and several other organs. The association between a malrotated liver and diaphragmatic hernia is extremely rare, with only a few cases previously reported in the literature. Surgery remains the definitive treatment, and the surgical approach depends on the size of the defect and the presence of significant complications.