IntRoductIon: the efficacy of Gamma Knife surgery (GKs) in local tumor control of non-secreting paragangliomas (PGLs) has been fully described by previous studies. However, with regard to secreting PGL, only one previous case report exists advocating its efficacy at a biological level. cAse RePoRt: the aims of this study were: 1) to evaluate the safety/efficacy of GKs in a dopamine-secreting PGL; 2) to investigate whether the biological concentrations of free methoxytyramine could be used as a marker of treatment efficacy during the follow-up. we describe the case of a 62-year-old man diagnosed with left PGL. He initially underwent complete surgical excision. thirty months after, he developed recurrent biological and neuroradiological disease; the most sensitive biomarker for monitoring the disease, concentration of plasma free methoxytyramine, started to increase. GKs was performed at a maximal marginal dose of 16 Gy. during the following 30 months, concentration of free methoxytyramine gradually decreased from 0.14 nmol/l (2*uRL) before GKs to 0.09 nmol/l, 6 months after GKs and 0.07 nmol/l at the last follow-up after GKs (1.1*uRL), confirming the efficacy of the treatment. Additionally, at 30 months there was approximately 36.6% shrinkage from the initial target volume. concLusIon: the GKs treatment was safe and effective, this being confirmed clinically, neuroradiologically and biologically. the case illustrates the importance of laboratory tests taking into account methoxytyramine when analyzing biological samples to assess the biochemical activity of a PGL. In addition, the identification of methoxytyramine as a unique positive biomarker could designate it for the monitoring of tumor relapse after treatments, including Gamma Knife surgery.