Abstract
PURPOSE: To report a rare case of congenital uveal malignant melanoma from Indian subcontinent in an infant with multiple cutaneous naevi and no distant metastasis. OBSERVATION: An 8 month old male child presented with proptosis and black discoloration in the right eye since birth. Enucleation of the right eye was performed and specimen sent for histopathology and immunohistochemical analysis. Results were strongly suggestive of a malignant melanoma of uvea. No metastasis was found on MRI scan of brain, chest and abdomen. Patient is on close follow up and will be subjected to metastasis workup every 6 months. CONCLUSION AND IMPORTANCE: This entity of congenital uveal malignant melanoma is rather rare with very few reported cases across the globe and needs further understanding of its correct line of treatment.