A male infant infected in utero with Epstein-Barr virus (EBV) demonstrated a syndrome of multiple congenital anomalies (micrognathia, cryptorchidism, central cataracts), hypotonia, thrombocytopenia, persistent monocytosis, proteinuria, and multiple areas of metaphysitis at birth. Lymphocytes were Epstein-Barr nuclear antigen (EBNA) positive (18%) and persisted in culture for three months. He had antibody to early antigen (anti-EA), IgM-viral capsid (anti-VCA), and EBNA (anti-EBNA) detectable at 22 days of age. All attempts to isolate infectious agents or to serologically identify other infectious causes for his syndrome were negative.