Melanotrichoblastoma (MTB) is an extremely rare benign skin tumor with follicular differentiation that clinically and dermoscopically resembles pigmented basal cell carcinoma (BCC), leading to frequent misdiagnoses. A 51-year-old man presented with a slow-growing blue-black nodule on the right canthus, present for 50 years, with recent central ulceration. Dermoscopy showed homogeneous pigmentation, atypical blue-white areas, and dilated vessels, suggestive of BCC. Histopathology revealed well-circumscribed dermal nests of basaloid cells with peripheral palisading and stromal pigment deposition, but absent retraction fissures typical of BCC. The lesion was completely excised and there was no recurrence after two years. This case highlights the diagnostic challenge of MTB, where histopathology is the gold standard and the absence of retraction fissures serves as a key feature to distinguish it from BCC. Increased awareness of MTB helps prevent misdiagnosis and enable conservative treatment.