The keratocystic odontogenic tumor (KCOT) is a benign developmental tumor with many distinguishing clinical and histologic features. Usually, multiple KCOTs occur as a component of nevoid basal cell carcinoma syndrome. The odontogenic myxoma is a rare benign tumor that represents about 3% of all odontogenic tumors. This article reports the case of mandible odontogenic myxoma with synchronous occurrence of multiple KCOTs, partial expression of nevoid basal cell carcinoma syndrome. A review of the international literature is also presented.