Objective: To determine the prevalence of midline nasofrontal lesions in a tertiary hospital of regional referral and it´s relationship with other malformations. Material and Methods: Study Design: Transversal retrospective. Male and female patients aged 1 month to 13 years of age with congenital lesions of the nasofrontal midline, treated at Children's Hospital from 1990 to 2005. Sample: was obtained by a non-probabilistic sample of consecutive cases. Through the Statistical Package for the Social Sciences (SPSS) version 13.0 which included descriptive statistics: median as a measure of central tendency and frequency distribution and proportion as a measure of dispersion. Results: a review of clinical records was conducted from 1990 to 2005, finding 38 of 67 records reviewed, 29 files were excluded because they correspond to the diagnosis of occipital encephalocele. Prevalence was 38 c...