Intrauterine ischemia of the limbs is a rare condition involving thrombosis and ischemia of the extremities before birth. The clinical presentation depends on the extremity affected as well as the timing of thrombosis. Very few cases with extremity hypoperfusion and gangrene due to intrauterine thrombosis have been reported in the literature, and therefore there is no consensus on the optimal therapeutic strategy for such cases. Presented here is a case of a newborn with intrauterine brachial arterial thrombosis of the right upper extremity who was successfully treated by a combination of recombinant tissue plasminogen activator, enoxaparin and collagenase application followed by surgery.