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Since granular cell tumor was first described by Abrikossoff in 1926, it has been known as a rare disease. The histogenesis of this tumor is still controversial, but the origin is thought to be from a Schwann cell. About one third of the tumors occur in the tongue, and uncommonly in the perianal region. We report a case of granular cell tumor that developed in the perianal region. The tumor grew slowly for 5 years and was removed by a local excision. This tumor showed positive staining with neuron-specific enolase (NSE).