Angiokeratoma is a cutaneous disorder that can either occur as solitary or multiple lesions, localized or generalized form, and is usually associated with underlying systemic metabolic disorder. Oral angiokeratoma (OAK) is rare and it has been observed as a part of generalized cutaneous angiokeratomas, whereas solitary OAK without systemic or cutaneous involvement is extremely rare. In this report, a rare case of solitary OAK of the ventral surface of the tongue in a 9-year-old male patient is described. Clinical differential diagnosis included pyogenic granuloma, haemangioma, lymphangioma, haematoma, lesions of melanocytic origin and malignant melanoma. Excisional biopsy was performed followed by a proper histopathological analysis. A definitive diagnosis of solitary angiokeratoma of the tongue was made according to the clinical and histopathological findings. The patient underwent further systemic examinations, and the results were non contributory. Follow up for 12 months after surgical excision, the patient remained asymptomatic, with no evidence of recurrence. The presented case of solitary angiokeratoma of the tongue is a rare disease entity. Accurate diagnosis of OAK entails thorough history taking, clinical and physical examinations, coupled with histopathology to rule out other benign and malignant clinical differential diagnosis.