The superficial palmar arch, formed by the branches of radial and ulnar arteries, supplies the blood to the hand. In cases of incomplete superficial palmar arch, the radial and ulnar branches fail to join. Any compromise to the ulnar blood supply in an incomplete superficial palmar arch can lead to subsequent gangrene. This baby presented with blackish discolouration and gangrene on the left hand, primarily affecting the little, ring, middle and index fingers. Timely intervention with antibiotics and heparinisation led to salvage of the ring, middle and index fingers, while the little finger underwent auto-amputation. On clinical exome sequencing, the baby was found to have DOCK6 mutation. This case highlights the importance of vascular involvement in DOCK6 mutation and the need for careful evaluation and management in cases of incomplete superficial palmar arch.
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