Purpose <p>To analyze the effect of systemic steroid treatment in children with benign acquired isolated sixth nerve palsy (BAISNP) regarding healing, recurrence risk and late sequelae.</p> Methods <p>A retrospective cohort design was used. The clinical database of a single medical center was reviewed for all children with BAISNP between 2016 and 2024. Data were collected as follows: demographics, age at initial presentation, complete eye and neurological examinations at presentation and every follow-up visit, neuroimaging and LP findings, treatments at presentation or for residual ocular misalignment, recovery periods and recurrences. Systemic steroids (1&#xa0;mg/kg/day oral prednisolone for 10 days) were recommended by certain physicians, to whom some patients were randomly assigned.</p> Results <p>Twenty-seven children were included in the study. Mean age at presentation was 2.6 ± 2.2 years with a follow-up of 2.0 ± 2.3 years. BAISNP completely resolved after 3.2 ± 3.1 months in all children. Nine children (33%) had a recurrence that resolved after 1.8 ± 0.7 months. Residual comitant esotropia (RCE) exceeding 10 prism diopters persisted in six children (22%) despite resolution of the abduction weakness. Three of them underwent surgery and none regained stereopsis. Fifteen children (56%) received systemic steroids upon presentation. Steroid treatment affected neither time to resolution nor recurrence rate. However, fewer children treated with steroids developed RCE (6% vs. 45%, <i>p</i> = 0.026). A multivariable analysis for RCE development found that only steroids had a statistically significant impact (β=−0.58, <i>p</i> = 0.006).</p> Conclusions <p>Systemic steroids reduce the rates of RCE when administered during an acute episode of BAISNP.</p>