A 12-year-old girl who underwent a splenectomy followed by heterotopic splenic autotransplantation for treatment of hypersplenism secondary to Gaucher disease at the age of 3 years presented with acute lower abdominal pain. Radiological investigations were highly suggestive of torsion of the autotransplanted splenic tissue, which was confirmed during subsequent laparotomy. This is the first known report of torsion of autotransplanted splenic tissue.