Opsoclonus myoclonus syndrome (OMS) / opsoclonus myoclonus ataxia syndrome (OMAS) is a rare central nervous system manifestation of COVID-19, but an increasing number of articles have reported patients in whom COVID-19 was complicated by OMS / OMAS. This narrative review aims at summarising and discussing current knowledge about the clinical presentation, diagnosis, treatment and outcome of SARS-CoV-2-associated OMS / OMAS. In total, 29 articles reporting 45 patients with SARS-CoV-2-associated OMS / OMAS were retrieved. Patient’s ages ranged from 2 to 88 years with three patients being children and the remainder adults. Gender was male in 32 patients and female in 13 patients. Opsoclonus was described in 29 patients, which was associated with myoclonus in 28 cases. Myoclonus was described in 43 patients, which was associated with opsoclonus and ataxia in 18 patients. OMS / OMAS was treated with steroids in 28 patients and with intravenous immunoglobulin (IVIG) in 15 patients. Clonazepam was given to 18 patients, levetiracetam to 13 patients, and sodium valproate to eight patients. Complete recovery was achieved in 12 cases and incomplete recovery in 22 cases. The authors conclude that diagnosing SARS-CoV-2-associated OMS / OMAS requires extensive neurological work up by clinical neurological examination, cerebral imaging with contrast medium, cerebrospinal fluid investigations and exclusion of differential causes. OMS / OMAS should not be missed as it usually responds favourably to steroids or IVIG.