This article presents a case with nevoid basal cell carcinoma syndrome (NBCCS) and an elongated styloid process. Basal cell carcinoma syndrome, also known as Gorlin-Goltz syndrome, is an autosomal dominant inherited syndrome manifested by multiple defects involving the skin, nervous system, eyes, endocrine system, and bones. Elongated styloid process or calcified stylohyoid ligament cause craniofacial or cervical pain. The actual cause of elongation of the styloid process or the calcification of the stylohyoid ligament is unclear. The cause of elongation of styloid process in this case may be the calcification induced by NBCCS. This report is the first case presentation of NBCCS with elongated styloid process. Elongated styloid process might be described as an anomaly of an NBCCS.