Pernicious anemia (PA) is an autoimmune disease secondary to chronic atrophic gastritis leading to vitamin B12 deficiency. Rarely, some patients may develop advanced hematological complications that mimic those of thrombotic thrombocytopenic purpura (TTP). Differentiating these conditions is crucial because they require different management. We present a case of a 68-year-old male who presented with generalized weakness, fatigue, and shortness of breath. This patient had anemia, thrombocytopenia, and a markedly deficient serum level of vitamin B12. The symptoms initially mimicked those associated with TTP, but the activity of ADAMTS 13 was normal. A diagnosis of pseudo-TTP has been made due to vitamin B12 deficiency resulting from PA with reactive thrombocytosis. Ultimately, vitamin B12 deficiency pseudo-TTP should be considered a differential diagnosis for therapy refractory TTP because of its different management strategies ranging from parenteral Vitamin B12 in PA patients with highly favorable outcomes to more advanced treatment with less favorable outcomes in TTP patients.