Granular cell tumors (GCTs) are rare soft tissue tumors that can occur in various anatomical locations, with less than 30 cases reported in the perianal region. These tumors typically present as firm, painless masses and may be misdiagnosed as more common anorectal conditions such as hemorrhoids or skin tags. Although most GCTs are benign, 1-2% can be malignant, making accurate diagnosis crucial. This case reports a 75-year-old female with a perianal GCT, who presented with a slow-growing, pruritic mass. On clinical examination, a 2 cm non-tender mass was noted at the 2 o’clock position. The patient underwent surgical excision, revealing a firm mass with superficial involvement of the internal anal sphincter. Histopathological examination confirmed a GCT with granular eosinophilic cytoplasm and positive staining for S-100. The patient had an uneventful recovery, with no recurrence at six months post-surgery. GCTs in the perianal region are rare and can be challenging to differentiate from other anorectal pathologies. Histopathological and immunohistochemical analysis, particularly S-100 staining, is essential for accurate diagnosis. Complete surgical excision remains the gold standard for treatment. Awareness of GCTs and their clinical presentation is critical to avoid misdiagnosis and ensure appropriate management, particularly in the perianal region where the differential diagnosis is broad.