Introduction. Antisynthetase syndrome (ASS) is a clinical entity recognized as the combination of auto-antibodies against an amino-acyl-tRNA synthetase and interstitial lung disease. Usually the patients also have myositis, dermatomyositis or polymyositis, where its degree of severity differentiates much among the patients. Other symptoms associated with ASS are mechanic hands, grotton’s papules, arthralgias, fever and Raynaud phenomena. Objective. This case-study follow a young female ASS-patient that because of end-stage pulmonary fibrosis was bilateral lung transplanted in August 2006. The follow-up time post-transplantation is 36 months. This is, know for us, the first reported cause of lung transplantation in a patient with ASS. Methods. Data on clinical and laboratory parameters, radiological lung imaging and pulmonary function tests were retrospectively collected from patient examinations. Results. The transplantation-procedure was uncomplicated, the same as the postoperative phase, except from some small rejections (grade 1-2) the first six months. After three years of follow-up her lung function is still stable with values within normal reference-levels for FVC, FEV1 and TLOC, and her quality of life is excellent. Conclusion. We report a cause of highly successful lung transplantation in a patient with ASS. Because of limited access to donors, high prevalence of contraindications in this patient group and the high risk of complications with this procedure, lung transplantation will still just be actually for a small part of patients with ASS.