The aim of this review was to assess the effectiveness of specific exercise types on pulmonary functions, aerobic and functional capacity in patients with ankylosing spondylitis (AS). A systematic search of Cochrane Database of Systematic Review, MEDLINE (EBSCO), Physiotherapy Evidence Database (PEDro), CINAHL (EBSCO), PUBMED, AMED, EMBASE (OVID) was conducted in January 2016. The outcome measures were spirometric measurements, chest expansion, 6 minute walk distance (6MWD), pVO(2), Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The search strategy was applied with limitation of date and language and this initial electronic search resulted in 143 relevant studies. After duplicates were removed, the titles and abstracts of 52 articles were screened. Of these, 14 full-text articles met initial criteria and were retrieved for review, with eight studies meeting final inclusion criteria. Both specific and conventional exercise groups showed significant improvements in BASDAI and BASFI scores (p < 0.05) in patients with AS, although there was no significant difference between two exercise groups. As for pulmonary functions, the specific exercise groups have greater improvements than conventional group in spirometric measurement, chest expansion (p0.05). Specific exercises are an effective adjuvant therapy to enhance cardiopulmonary functions in patients with AS; therefore, it is assumed that in addition to the medical treatments, specific exercise therapy might reduce the cardiopulmonary complications related with AS.