The increasing number of reports of disseminated BCG infection (BCG-itis) is very serious, and is almost always in children with immunocompromising conditions such as HIV, SCID, or with another severe form of congenital immunodeficiency. Vaccination at birth is a constant element of vaccination programmes in Central and Eastern Europe, due to the high prevalence of tuberculosis. Difficulties in the diagnosis and therapy of BCG infections in primary immunodeficiency patients, hospitalised in the Department of Immunology, Children’s Memorial Health Institute in Warsaw, during the last 25 years have recently been published in Emerg Infect Dis 2007; 13(5): 799. Based on our experience, we would like to propose a set of novel criteria for diagnosis and prophylaxis, and therapeutic guidelines for BCG infection.