This article describes a case of odontogenic keratocyst (OKC) in a 1 year and 7 month old girl who presented such a lesion mimicking an eruption cyst. To date, only one well-documented OKC occurring in a patient under 5 years old has been reported and it was thought to be associated with nevoid basal cell carcinoma syndrome (NBCCS). In our OKC case, the cyst was totally enucleated. No evidence of recurrence and NBCCS was found after a 4-year follow-up. The development of involving tooth in a growing child and the histogenesis of OKC are discussed in this article.

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