Leptomeningeal carcinomatosis is an extremely rare, but devastating complication in pancreatic cancer patients with a poor prognosis despite multimodal treatment. We present a 51-year-old male patient with the very rare condition of leptomeningeal carcinomatosis originating from pancreatic cancer. He presented to our hospital with severe headache and neck stiffness 30 months after systemic chemotherapy. Cerebral and spinal MRI as well as cerebrospinal fluid examination confirmed the diagnosis of leptomeningeal carcinomatosis. The patient responded to gemcitabine plus nab-paclitaxel in terms of elimination of tumor cells from the CSF and concurrent clinical improvement for 3 months. The observed findings suggest that the combination of gemcitabine plus nab-paclitaxel is potentially effective in affected cerebrospinal fluid of pancreatic carcinoma patients.