PURPOSETo report association of herpes simplex virus-type 1 (HSV-1) in four cases of congenital cataract.DESIGNProspective interventional case series.METHODSFour infants younger than 12 months, presenting with unilateral or bilateral congenital cataract, were included. The cases were clinically evaluated by the pediatric ophthalmologist. The lens aspirates collected at the time of cataract surgery were processed for HSV-1 culture in rabbit corneal epithelial (SIRC) cell line and for HSV-1 DNA by polymerase chain (PCR). The sera of the children and the mother were tested for HSV-1 immunoglobulin (Ig) M and IgG by enzyme linked immunosorbent assay (ELISA).RESULTSHSV-1 was isolated in tube cultures in three of four lens aspirates, and all four lens aspirates were positive for HSV-1 DNA by PCR. Serum HSV-1 IgM was positive in all babies and in three cases HSV-1 IgM was positive in the mother's serum.CONCLUSIONBased on a computerized literature search, we believe this may be the first report of HSV-1 associated congenital cataract.