We present the correlative diagnostic imaging and histological findings in a case of multiple odontogenic keratocysts (OKC) associated with the Nevoid Basal Cell Carcinoma Syndrome (NBCC). Whilst CT displayed aspects of bone morphology not visible on the plain films, contrast-enhanced MR provided the essential macropathological detail, including focal wall-enhancement and iso-intense intraluminal soft-tissue masses which correlated with the histological findings of focal inflammatory ulceration of the cyst lining, orthokeratosis and cell debris. The features identified by these combined imaging techniques are helpful in distinguishing the OKC from other cysts or neoplastic lesions and can therefore alter surgical management.