Ankylosing spondylitis (AS) can be associated with extra-articular manifestations, among which we find pulmonary disorders. Fibrosis of the pulmonary apices is seen in up to 30% of the cases, and cyst formation is less common, being seen in advanced cases. Colonization of those cavities is a rare complication. A patient with a diagnosis of AS since 1998 with axial involvement and history of pulmonary tuberculosis treated in 2002 and 2007, developed bilateral aspergillosis of the pulmonary apices associated with zygomycosis and Actinomyces spp. The patient had been hospitalized to investigate complaints of weight loss, nocturnal diaphoresis, productive cough, and lesion in both lung apices. He was submitted to right upper pulmonary lobectomy after identification of a fungus ball on chest X-ray and CT scan, which was confirmed by a fibrobronchoscopy and biopsy for anatomopathological exam. The patient evolved without expansion of the right lung and underwent another fibrobronchoscopy that suggested occlusion of the middle lobar bronchus. Repeat thoracotomy did not confirm the findings of the last fibrobronchoscopy, but the right lung failed to expand. The patient developed septic shock refractory to treatment and died.