An 11-year-old Japanese female having acanthosis nigricans associated with Crouzon syndrome is reported. Crouzon syndrome is a craniostenotic craniofacial malformation associated with premature closure of selective calvarial sutures, exophthalmos, maxillary hypoplasia, and a beak-shaped nose. It is an autosomal dominant inherited disorder. Crouzon syndrome is one of the syndromes which may be associated with acanthosis nigricans. The association of acanthosis nigricans with Crouzon syndrome is assumed to be a rare abnormality, although the true frequency is uncertain. We have reviewed the reported cases of acanthosis nigricans associated with Crouzon syndrome and characteristics were discussed.