Aim: This report aims to discuss and review the diagnosis and management of acute acquired comitant esotropia (AACE) in children and to add several cases to the limited literature available on this unusual condition in the pediatric population. Materials and methods: We present two cases of AACE with large-angle deviations that were investigated and followed-up according to current recommendations. Both cases required strabismus surgery for AACE, but different procedures were chosen, with good postoperative results. Results: Unilateral recession of the medial rectus and resection of the lateral rectus (R&R) were performed in one case and bilateral medial rectus (MR) recession in the other, with resolution of the diplopia and full recovery of binocular vision. Discussion: Although isolated AACE is usually benign, studies have reported the presence of intracranial disease in up to 10% of cases, making it a potential first sign of an underlying serious pathology. Therefore, AACE should be investigated as a medical emergency and neuroimaging should be performed in all patients with unclear onset of AACE, as well as in those with associated neurological symptoms, such as headache, cerebellar imbalance, weakness, or nystagmus. Conclusion: Acute acquired comitant esotropia (AACE) is an infrequent type of esotropia that usually appears in older children. It is characterized by esotropia and diplopia with acute onset. Neurological examinations and neuroimaging should be performed to exclude any potential intracranial disease. Treatment of AACE without underlying neurological disease is focused on managing the diplopia and resolving the esotropia. Strabismus surgery has good motor and sensory results and can successfully restore good binocular function. Abbreviations: AACE = Acute acquired comitant esotropia, LR = lateral rectus, MR = medial rectus, PD = prism diopters, R&R = recession and resection, BSV = binocular single vision, PAT = prism adaptation test.