The reporting case is a 27 year-old woman. She was admitted with complaints of clumsy gait and dysesthesia over her upper limbs and spasticity in the legs. The investigations revealed a long segment cervical intramedullary tumor, associated with whole cord syringomyelia. The superior part of syringomyelia image intensity matched intramedullary lipoma completely. C1–C5 intramedullary tumor was resected grossly under microscopic view, and a fat-containing liquid exited the syringomyelia cavity at its superior pole. After a transient paresis just following the surgery, neurological condition was improved. The follow-up imaging showed that the fatty syringomyelia reduced in dimensions. Conclusion. According to this case and another similar case due to instability, it appears that as a rare phenomenon, syringomyelia liquid content sometimes could have fatty droplets which could mimic intramedullary lipoma with an unknown mechanism. It seems these fat droplets in syringomyelia would not change its natural history.