Myelodysplastic syndrome (MDS) is a clonal hematopoietic disorder that often results in progression to acute myeloid leukemia (AML), particularly when additional genetic abnormalities are present, such as monosomy 7. Treatment options for this condition are limited. Spontaneous remission has occurred in a small number of cases. In the present case, a previously healthy 3-year-old boy diagnosed with MDS and monosomy 7 achieved spontaneous remission without intervention 30 months after initial diagnosis. Such findings highlight the need to monitor patients closely for evidence of spontaneous remission.