A 15 years old male with a primary diagnosis of Wiskott Aldrich syndrome presented a laryngeal B cell lymphoma associated with Epstein-Barr virus. A chemotherapy and a radiotherapy were started in association with an endoscopic debulking of the tumor. The child died of respiratory failure secondary to a lung infection. The incidence of NHL (non-Hodgkin's lymphoma) of laryngeal origin in infants is extremely low and to our knowledge it has never been found in Wiskott Aldrich syndrome.