Abstract
Anti-synthetase syndrome (ASS) is a rare autoimmune disease whose hallmark consists of the presence of autoantibodies directed against aminoacyl transfer RNA (tRNA) synthetases thus making it a distinctive pathology of the broad spectrum of idiopathic inflammatory myopathies (IIM). Clinically, lung involvement in the form of interstitial lung disease (ILD) and myositis are typical findings. Inflammatory myopathies should always raise the suspicion of an adjancent tumoral process and therefore prompt the clinician to perform a thorough screening. We present the case of a 55 year old caucasian male who was diagnosed with amyopathic ASS and a clear cell renal carcinoma (Grawitz tumor), along with the consequent therapeutic challenges.