We report a case of intratumoral fat in neurofibroma and coexistance of eccrine hidrocystoma on the occipital area of the scalp for one year duration. There are several diseases showing fatty change histopathologically. Among them, few cases of neurofibroma showing fatty change had been reported. The mechanism of fatty change is unclear, but several hypotheses are proposed. Here we report a case of neurofibroma showing fatty change and coexistence of eccrine hidrocystoma, and discuss the pathomechanism of fatty change and its relationship with disease.