A preterm, very low birth weight infant was born to a mother with early lat
ent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU
of benzathine penicillin The infant had clinical, laboratory, and radiogra
phic abnormalities consistent with congenital syphilis, ie, a Venereal Dise
ase Research Laboratory test titer that was fourfold greater than was the m
aternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonit
is, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examin
ation showing a reactive Venereal Disease Research Laboratory test, pleocyt
osis, and elevated protein content. The infant died on the third day of lif
e, and an autopsy revealed an evolving gumma of the anterior pituitary. Imm
unoglobulin M immunoblotting of serum and CSF was positive, and polymerase
chain reaction detected Treponema pallidum DNA in endotracheal aspirate and
CSF. This case highlights the pathologic abnormalities observed in congeni
tal syphilis and focuses on the rare finding of an evolving anterior pituit
ary gumma. Furthermore, it documents the failure of maternal syphilis treat
ment during the last 4 weeks of pregnancy to cure fetal infection and suppo
rts the recommendation that all infants born to mothers with syphilis treat
ed during the last 4 weeks of pregnancy should receive penicillin therapy.