Veno-occlusive disease (VOD) of the liver is characterized by jaundice, painful hepatomegaly, and retention of fluids. VOD is a severe complication in allogeneic stem cell or bone marrow transplantation. Additionally, the disease has been described in children suffering from nephroblastoma or rhabdomyosarcoma, treated with intense chemotherapy. Recently, VOD has been shown to be a complication in the treatment of myeloid leukemia with anti-CD33 linked to calicheamicin. We report the unusual case of a 21-year-old male patient with Marfan syndrome, diagnosed of acute lymphoblastic leukemia, who developed severe VOD during induction therapy after a single application of 2 mg vincristine. We speculate that coincidence of Marfan syndrome and application of induction chemotherapy might favor the disease in our patient.