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Background: The co-occurrence of Rathke cleft cysts (RCCs) and meningiomas in the sellar and parasellar regions represents an exceedingly rare clinical entity. Achieving maximal resection through a single operative approach while minimizing adverse events is challenging, often necessitating multiple surgical approaches, as suggested by previous reports., Observations: The authors report the case of a 49-year-old female with a history of kidney transplant who presented with headaches and was diagnosed with coexisting RCC and meningioma in the sellar and planum sphenoidale regions, respectively. Given the prolonged, refractory nature of her symptoms and based on her treatment preferences, a two-stage surgical approach was planned. This involved an endoscopic transnasal transsphenoidal approach followed by a right craniotomy to achieve maximal tumor resection while minimizing potential complications. No residual disease or tumor remnants were present at the 6-month follow-up. The pituitary gland was preserved, and the patient's preoperative symptoms had fully resolved., Lessons: The preoperative diagnosis of concurrent skull base tumors is essential for effective management planning and determining the optimal surgical approach. The proximity of these tumors to critical neurovascular structures necessitates meticulous surgical planning to minimize adverse effects while ensuring maximal tumor resection. https://thejns.org/doi/10.3171/CASE24551.