Type B insulin resistance syndrome (TBIRS) has not been previously reported in Arab populations. We report a case of TBIRS in an Arab patient with mixed connective tissue disease (MCTD). Investigations revealed a clinical condition marked by positive anti-insulin receptor and ribonucleoprotein antibodies. The patient presented with severe hyperglycemia, weight loss, arthralgia, and acanthosis nigricans. He was managed successfully with an intensive pulsed combination regimen of cyclophosphamide and plasmapheresis augmented with high doses of prednisolone, which normalized his blood sugar levels without insulin therapy. MCTD was controlled by hydroxychloroquine. During the course of his treatment, the patient developed diabetic ketoacidosis secondary to the gluteal abscess, leukopenia, and recurrent hypoglycemia. Here, we showed that immunosuppressive agents and plasmapheresis can induce remission of TBIRS and can be used to normalize the blood sugar levels of Arab patients with this condition.