Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by biallelic mutations in the ADAMTS13 gene, leading to recurrent microangiopathic hemolytic anemia and thrombocytopenia. Due to its rarity, cTTP is frequently misdiagnosed as immune thrombocytopenia (ITP) or other hematologic disorders, delaying appropriate management. Case Report: We describe a 16-year-old Indian girl with a history of multiple episodes of thrombocytopenia, anemia, and heavy menstrual bleeding. Initially diagnosed and treated as ITP, she presented with left-sided hemiparesis, thrombocytopenia, and anemia. Laboratory investigations revealed severely deficient ADAMTS13 activity (2.7%) with absent ADAMTS13 antibodies, suggestive of cTTP. Genetic analysis confirmed compound heterozygous mutations in ADAMTS13 (c.2317C>T, p.Gln773Ter in exon 19 and c.1337T>C, p.Met446Thr in exon 12). The patient was treated successfully with fresh frozen plasma (FFP) infusions and maintained on prophylactic plasma therapy every 2-3 weeks, with reduced volume to minimize transfusion-related complications. Conclusion: This case underscores the importance of considering cTTP in unexplained recurrent thrombocytopenia and anemia, especially in cases with neurological involvement. Early diagnosis with ADAMTS13 activity testing and genetic confirmation is crucial to prevent life-threatening complications and optimize long-term management.