DESCRIPTIONIntrauterine gangrene of an extremity is a rare problem. Lower extremity gangrene is even rarer. 1 We present a case of lower extremity gangrene in combination with Tetralogy of Fallot (TOF) and postaxial polydactyly of the hand. A live male infant was delivered by caesarean section. Delivery occurred at the 34th week of gestation due to meconium aspiration and intrauterine growth retardation. The mother was 35 years of age and had three healthy children. She had been diagnosed with gestational hypertension during this pregnancy. The newborn patient weighed 1740 g (10th centile) and his length was 43 cm (25th centile). After birth, he developed respiratory distress and was intubated. Examination revealed postaxial polydactyly with palpable phalanxes on the left hand, with an attached necrotic soft tissue mass (figure 1), as well as lower extremity gangrene with a visible demarcation line on the left middle thigh (figures 2 and 3).Blood test results were normal except for a low platelet count of 73 000/mm 3 (normal range 130 000-510 000/mm 3 ), elevated D-dimer of 1154.88 ng/mL (normal range <500 ng/mL), low antithrombin III activity of 55% (normal range 70-125%) and low protein-S activity of 22% (normal range 33-93%).Doppler ultrasonography revealed blocked arterial flow below the level of the tibioperoneal trunk. The main, deep and superficial femoral and popliteal arteries were patent. The patient also had TOF, which was diagnosed by a cardiologist. A transfemoral amputation and polydactyly excision were performed on the third day after birth. The histopathological investigation of the tissue blocks from the amputation material was not helpful in identifying the aetiology. On the 32nd day after birth, the patient was operated for TOF. He died on the seventh postoperative day due to cardiac arrest.