Necrotizing fasciitis (NF) is an uncommon destructive disease and fatal infection of the subcutaneous tissue. The literature includes a limited number of NF cases in rheumatic disease such as rheumatoid arthritis and systemic lupus erythematosus (SLE). We report a 40-year-old patient who had complicated with NF during treatment with corticosteroid and azathioprine for overlap syndrome of systemic sclerosis and SLE. She underwent urgent surgical debridement and internal drainage with antibiotics and had complete recovery from NF. To our knowledge, this is the first case of NF developed in a patient of overlap syndrome with diffuse type of systemic sclerosis and SLE and suggests that NF can be a very rare cutaneous manifestation of this disease.