Nevoid basal cell carcinoma syndrome (NBCCS), an autosomal dominant disorder with a high degree of penetrance and variable expressivity, is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. More than 100 minor criteria have been described but two major and one minor criteria or one major and three minor criteria are necessary for the diagnosis. In this report we present a 8 year old girl affected by NBCCS showing an uterus bicornis, an hitherto unreported association. However further researches are needed to confirm the association between NBCCS and mullerian fusion defects and to assess the hypothesis that focuses on chromosome 9 the mutant gene for NBCCS and fusion defects of female genital tract
