Summary:Background.:Glanzmann thrombasthenia (GT) is a rare bleeding disorder characterized by absence or dysfunction of the glycoprotein (GP) IIb-IIIa complex on the surface of platelets.Case.:A 5-year old boy, with recurrent severe nose bleeds and frequent hematemesis repeatedly leading to circulatory insufficiency for which intensive care (IC) admissions were necessary. Underlying disorder was a severe inherited Glanzmann thrombasthenia. After developing alloantibodies against GPIIb-IIIa complex on donor platelets (anti-HPA-1 and anti-HPA-3) treatment of bleeding was possible only by activated rFVII. Because of the severity of the bleedings and limited treatment options the patient was eligible for stem cell transplantation (SCT) with bone marrow from his HLA-identical sister. This transplantation was successful and uncomplicated.Conclusion.:Allogeneic stem cell transplantation is curative for Glanzmann thrombasthenia. In case of Glanzmann thrombasthenia with a complicated course this treatment can be considered when a compatible donor is available.