New onset refractory status epilepticus (NORSE) is a clinical presentation in patients, not a specific diagnosis characterized by the occurrence of a prolonged period of refractory seizures. NORSE is the presenting clinical feature in some patients with paraneoplastic etiology. Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It presents as a subacute disease with symptoms like seizures, cognitive dysfunction, irritability, hallucination, and short-term memory loss. In our study, we reported a case of a 30-year-old woman with PLE associated with craniopharyngioma and serum anti-Hu antibodies. The patient presented with NORSE. Initial therapy with antiepileptic drugs failed to control seizures. The patient presented with short-term or recent memory loss, cognitive dysfunction, and behavioral changes (anger/apathy) for the last three months, suggesting a rapidly progressing dementia. Baseline investigations (blood counts, renal function, thyroid function, liver function, serum B12/folate level, serum cortisol, and urinalysis) were normal, and she had no background comorbidities. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) revealed a sellar/suprasellar lesion suggestive of craniopharyngioma. The tumor was successfully removed and repeat imaging after surgery was clear and did not result in further seizures. Now the patient is on follow-up with tapering doses of prednisolone. This is a rare condition; we treated our patient successfully via surgical resection and postoperative chemotherapy. Also, we considered that her new onset refractory epilepsy was associated with her craniopharyngioma. Hence a high degree of suspicion is required, which can be guided by clinical findings and imaging.