L.W. is a 48-year-old right handed female with a scleroderma overlap syndrome (scleromyositis) manifested by systemic sclerosis (skin and gastrointestinal involvement) with a predominantly proximal necrotizing myositis. It is likely that this is a rare polymyositis/scleroderma overlap or anti-synthetase syndrome. She is persistently antibody negative for U1RNP, PmScl, Ro, La, Jo-1 and Ku antibodies. Antibodies to smooth muscle and skeletal muscle are also negative. Her symptoms were treatment resistant to immunosuppression until B cell depletion with rituximab. We discuss her course and prognosis. The patient presented in 2008 with progressive gastrointestinal symptoms with malabsorption, eventually requiring total parenteral nutrition. Total parenteral nutrition resulted in improved general condition with weight gain. She was being treated with combination of methotrexate and prednisolone when she developed insidiously progressive proximal weakness, with parallel progression of skin thickening. Peak creatine kinase (CK) was recorded at 1800u/L. There was no improvement in muscle strength with steroid reduction. Electromyogram in 2010 suggested a proximal predominant necrotizing myositis. We postulate she has a scleroderma overlap syndrome. She was treated with rituximab in January 2012. L.W. was monitored clinically with CK levels. B cell depletion was confirmed with CD20 count of <1%. Initially, she had severe disability with Medical Research Council (MRC) grade 3 upper and lower limb power proximally. She improved with MRC grade 4/4+ power proximally. CK normalised. The skin also improved, from widespread trunk and limb involvement, to minor skin thickening in the hands. Prednisolone dose reduction was successful over 2012 to 2013. Methotrexate was also reduced from 20mg weekly to 15mg. At 18months following rituximab therapy, L.W. had increase in CK to 330u/L, with early B cell recovery demonstrated on lymphocyte subsets. Rituximab therapy for systemic sclerosis is increasingly utilized; however this is a unique case of a scleroderma-myositis overlap syndrome achieving impressive results with B cell depletion and subsequent redosing.