Autoimmune hemolytic anemia (AIHA) secondary to mediastinal teratoma is a very rare clinical entity. They can be primary or secondary to any infections, malignancy, etc. Yet at times, there could be a delay in the diagnosis. Here we present a rare presentation of mediastinal teratoma and probably this is the first from India, based on our literature review using PubMed and Scopus as search engines with MeSH (Medical Subject Headings) words “mediastinal teratoma AND India AND hemolytic anemia.” A 26-year-old male with cough and effort intolerance was diagnosed with AIHA which was triggered by a mediastinal teratoma. He had difficulty in preprocedural blood transfusion due to incompatibility which needed high-dose methylprednisolone to suppress the immune system. Due to prior usage of pulse steroids, we had the challenge of ruling out hematological malignancy, which was done by bone marrow examination and positron emission tomography (PET) scan of the whole body. AIHA resolved completely with open thoracotomy and excision of the tumor.