Idiopathic inflammatory myopathy (IIM) with antimitochondrial M2 antibody (AMA-M2) has been associated with distinct clinical characteristics. In this study, we explore the magnetic resonance imaging (MRI) findings of the muscles of the lower extremities in AMA-M2-positive IIM to gain more insight. MRI of 22 lower extremity muscles was retrospectively evaluated in 14 patients with AMA-M2-positive IIM and 37 age- and sex-matched patients with AMA-M2-negative IIM. Muscles with inflammatory edema and fatty infiltration were assessed according to the Stramare and Mercuri criteria. Patients with AMA-M2-positive IIM had significantly higher incidence of MRI involvement with fatty infiltration in five lower extremity muscles, namely the adductor magnus (AM) (13/14 VS 14/37, p < 0.001), semimembranosus (SM) (13/14 VS 17/37, p = 0.002), biceps femoris (BF) (12/14 VS 15/37, p = 0.004), soleus (13/14 VS 23/37, p = 0.041), and the medial head of the gastrocnemius (Gastroc M) (13/14 VS 17/37, p = 0.002) than patients with AMA-M2-negative IIM. Furthermore, the severity scores of fatty infiltrations of the above five muscles in AMA-M2-positive IIM were significantly higher than those in patients with AMA-M2-negative IIM (p < 0.001). Severe fatty infiltrations of the AM, SM, BF, soleus, and Gastroc M in the posterior muscles of the lower extremities are dominant MRI features in our patients with AMA-M2-positive IIM. This unique muscle MRI character may be a helpful indicator in clinical practice for patients with AMA-M2-positive IIM. Key Points • Striking involvement and prominent fatty infiltrations of five lower extremity muscles (adductor magnus, semimembranosus, biceps femoris, soleus, and the medial head of the gastrocnemius) are interesting MRI performances. • Severe fatty infiltrations in the posterior muscles of the lower extremities are dominant MRI features in AMA-M2-positive IIM. • This unique muscle MRI character may be very helpful for the diagnosis of the AMA-M2-positive IIM.