Background: Ocular toxocariasis is considered a parasitic disease of major socioeconomic importance. In spite of the high prevalence of human toxocariasis (up to 84%) among Egyptian patients, the incidence of ocular toxocariasis is underestimated. The recognition of this neglected disease would be the initial step to overcome it. Thus, this review gave updated information on the pathogenesis, clinical manifestations, diagnosis, and treatment of ocular toxocariasis. Results: Ocular toxocariasis is an important cause of unilateral vision impairment mostly in children and always in the differential diagnosis of retinoblastoma. This disease exhibits various manifestations such as posterior pole granuloma, peripheral granuloma, or chronic endophthalmitis. Diagnosis of ocular toxocariasis can be carried out by the ophthalmic examination and immunodiagnostic methods to reveal the specific antibodies in serum and ocular fluids. In addition, molecular diagnosis, medical imaging techniques, and histopathologic observation of Toxocara larva in the surgically obtained specimens can be performed. Ocular toxocariasis can be treated either medically or surgically. Regarding medical treatment, the ophthalmologists prefer to use steroids and anthelminthic drugs; however, there are no standardized parameters for doses, duration, and route of administration. Conclusion: Clinical suspicion plays a leading role in the diagnosis of ocular toxocariasis, but always with other diagnostic methods. Accurate diagnosis and prompt treatment can minimize ocular morbidity.