Congenital anterior staphyloma, a rare developmental anomaly of the eye, and dermoid cysts, benign congenital choriostomas, are uncommon ocular conditions with distinctive clinical presentations. We herein present a case report of a 2-year-old girl with a painless right corneal mass present since birth, which gradually enlarged. Clinical examination revealed a congenital anterior staphyloma with a coexisting pedunculated dermoid attached to the cornea at the inferior limbus. Surgical excision was performed, and histopathological examination confirmed the diagnosis of a dermoid cyst. The complex interplay between developmental abnormalities and ocular manifestations poses diagnostic and management challenges. While the etiology of congenital anterior staphyloma remains unclear, theories suggest intrauterine ulceration or defective anterior mesoderm differentiation. Early developmental errors leading to metaplastic transformations or sequestration of pluripotent cells during embryonic development could have resulted in the pedunculated dermoid. Management strategies vary depending on the extent of ocular involvement, with enucleation being necessary in cases of severe visual impairment.