Pycodysostosis is a genetic autosomal rare disease with an incidence of 1:1.7 million births; the pathophysiology of the disease is related to mutation of cathepsin K gene. Sleep apnea, respiratory difficulties because of chest and oral abnormalities may cause a challenge to the anesthetist during intubation and/or mechanical ventilation. In this case report we will discuss a case of pycodysostosis with a difficult airway.