Objective Anterior temporal lobectomy (ATL) surgery for epilepsy is commonly associated with post-surgical impairments in memory and language abilities, specifically episodic memory and confrontational naming. Less is known regarding outcomes of patients with atypical language dominance. Method Casey is a 19 year-old, ambidextrous, male with a history of remote symptomatic medically intractable focal epilepsy secondary to right inferior temporal encephalomalacia. Casey underwent right anterior temporal lobectomy and resection of mesial structures including part of the hippocampus. Results Casey’s pre-surgical neuropsychological evaluation revealed overall intellectual functioning in the average range with relative strengths in perceptual reasoning skills and weaknesses in attention, executive control, confrontational naming, and verbal fluency. Casey’s pre-surgical cognitive profile and functional MRI were suggestive of bilateral language organization. At post-surgical evaluation, Casey demonstrated strong overall cognitive abilities, basic reading, visual–spatial skills, and visual memory, as well as notable improvements in his processing speed and visual-motor integration. He demonstrated significant improvement in verbal working memory, verbal fluency, and contextual verbal memory. Casey continued to show significant weaknesses in word finding, phonetic verbal fluency, and rote verbal learning and memory, and milder weaknesses in aspects of executive functioning. Casey continued to demonstrate mood difficulties. Conclusions The current case provides evidence of continued mild deficits in traditional dominant hemisphere skills including rote verbal memory and focal language abilities, in a patient with a history of mixed language dominance, following right hemisphere ATL surgery. This case further highlights the importance of pre-surgical neuropsychological evaluation and fMRI in patients undergoing right ATL surgery.