Objective To assess the efficacy and safety of tacrolimus in combination with corticosteroids in patients with immune-mediated necrotising myopathy. Methods The medical records of 20 hospitalised patients with immune-mediated necrotising myopathy (IMNM) who had received tacrolimus combined with oral prednisone from January 2014 to August 2017 were retrospectively reviewed. The recruited patients were shifted to the combined therapy because they failed to respond well to monotherapy with oral prednisone. The clinical efficacy during an average follow-up of 21 months (range, 14-24 months) was assessed by evaluating the changes of serum creatine kinase (CK) levels, the Medical Research Council (MRC) grading of the weakest muscle groups and dosage of oral prednisone. Adverse effects were monitored to assess the safety of tacrolimus. Results After starting tacrolimus, most of the 20 patients showed significant improvement in muscle strength and remarkable decline in serum CK levels at the follow-up points (p<0.0001). In addition, the daily dosage of prednisone was statistically significantly reduced (p<0.0001) after the combination therapy. No serious adverse events attributable to tacrolimus occurred in the patients. Conclusion Early co-administration of tacrolimus with corticosteroid promoted the remission and recovery of patients with IMNM and seemed to be a relatively safe treatment programme for physician managing immune-mediated necrotising myopathy.