Palatal Congenital Melanocytic Nevus With Unusual Clinical Feature: A Rare Case Report With Literature Review

Congenital melanocytic nevi are uncommon benign lesions that primarily involve the skin and are exceedingly rare within the oral cavity, where their atypical presentation may pose a diagnostic challenge due to clinical overlap with other pigmented lesions. We report a case of an intramucosal palatal congenital melanocytic nevus (CMN) in a 34-year-old woman who presented with a painless nodular lesion arising from a lifelong pigmented area of the palate. Histopathological examination demonstrated a benign proliferation of nevus cells with theques formation. The deepest nevus cells showed proximity to the adjacent minor salivary gland lobules. The lesion was managed by complete surgical excision, with uneventful healing at 6-month follow-up. A comprehensive review of the literature identified only 11 reported cases of intraoral CMNs, confirming the palate as the most frequently involved site and a marked female predominance. This case highlights the critical role of correlating clinical history with histopathological findings to achieve an accurate diagnosis.