Distichiasis, an extra row of eyelashes emerging from meibomian gland orifices, occurs due to the metaplastic transition of sebaceous glands into the pilosebaceous unit. It can present congenitally, such as in lymphedema distichiasis syndrome, or secondary to acquired conditions, such as cicatrizing conjunctivitis, trachoma. This review summarizes the etiology of distichiasis, its presentation, the evolution of various surgical techniques, and their outcomes in human and animal eyes. The published literature has focused on the different treatment modalities and their outcomes; the etiopathogenesis of this condition remains elusive. Truncating mutations (missense, frameshift, and nonsense) in the Forkhead family gene FOXC2 are involved in the distichiasis–lymphedema syndrome. The treatment options are no different for congenital versus acquired distichiasis, with no specific available algorithms. Acquired distichiasis in cicatrizing ocular surface diseases is difficult to manage, and existing treatment options offer success rates of 50%–60%. The outcomes of electroepilation or direct cryotherapy are not as good as surgical excision of distichiatic lashes after splitting the anterior and posterior lamella under direct visualization. The marginal tarsectomy with or without free tarsoconjunctival graft has shown good results in eyes with congenital and acquired distichiasis. The details of differences between normal and distichiatic lash, depth, or course of distichiatic eyelashes remain largely unknown. Studies exploring the distichiatic eyelash depth might improve the outcomes of blind procedures such as cryotherapy or radiofrequency-assisted epilation.