We report a case of neonatal allo-immune thrombocytopenia due to fetomaternal human platelet antigen (HPA)-1 incompatibility. Anit-HPA-1b antibodies were detectable in maternal serum. Repeated treatment of the infant with high-dose IgG effectively raised platelet counts transiently, but the patient remained thrombocytopenic for more than 6 weeks. Serological and DNA analysis revealed that the mother was homozygous HPA-1a and the father homozygous HPA-1b.