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Case: Malignant granular cell tumors are uncommon soft-tissue tumors. We describe a granular cell tumor that presented as a benign hand nodule diagnosed by biopsy and image. The patient refused local excision. After 15 years, the patient came back because of symptomatic growth, and a malignant granular cell tumor was diagnosed, with metastatic disease. Despite surgical disarticulation and adjuvant treatment, she died after 8 months. The pathological examination revealed the tumor had progressed to malignant behavior. Conclusions: Our findings highlight the importance of a closer follow-up. Although unusual, we must be aware of the possibility of a change of behavior from benign to malignant granular cell tumor.