PURPOSE: This study aimed to evaluate odontogenic keratocysts associated with basal cell nevus syndrome (BCNS) using computed tomography (CT) and magnetic resornance imaging (MRI) including diffusion-weighted imaging (DWI) and compare them with sporadic cases. MATERIALS AND METHODS: This study investigated 17 outpatients who underwent panoramic radiography, CT, and MRI between August 2012 and January 2021. Five of these patients had BCNS had 16 odontogenic keratocysts, for which the authors recorded detailed findings. DWI analysis compared the apparent diffusion coefficient (ADC) values of odontogenic keratocysts in patients with BCNS and sporadic cases. The Mann-Whitney test was used to analyse bivariate statistics. RESULTS: Patients with BCNS had an average of 3.2 lesions in the jaw. On DWI, the ADC value ranged from 0.58 to 2.66 × 10 CONCLUSION: This study presented detailed imaging findings of odontogenic keratocysts in patients with BCNS. Furthermore, the authors revealed a wide range of ADC values for BCNS-associated odontogenic keratocysts.