A 4-year-old female presented to our institution in April 2021 with a four-month history of medication resistant hypertension, intermittent diaphoresis, flushing, tachycardia, wringing of her hands and feet due to reported pruritis, sleep disturbances, new onset seizures and generalized developmental regression. Comparison of MRI brain between March 2021 and April 2021 revealed global cerebral atrophy. Her EEG was unremarkable. She was found to have a positive LGI1-IgG serum antibody, as well as elevated VMA (52.7) and HVA (39.3). Her CT, CTA, and MRI of the abdomen revealed a 3 cm right renal mass later resected and identified as a benign cystic nephroma. She received 30 mg/kg/day of IVMP daily for 5 days and 2 g/kg intravenous immunoglobulin (IVIG). She subsequently received 30 mg/kg IVMP weekly for 4 weeks, rituximab 500 mg/m2 twice and 1 g/kg IVIG monthly for 6 months. At the 4-month clinic follow up, she returned to baseline aside from the need for one antihypertensive and low dose gabapentin for neuropathic pain management.