Metastasis in the pineal region is a rare condition. To best of our knowledge, there is no case report of isolated pineal metastasis secondary to acute lymphocytic leukemia (ALL). The aim of this study is to show the pineal gland involvement of ALL in a case for the first time in the literature. A 25-year-old male patient diagnosed with ALL 2 years ago presented with headache and visual impairment. Brain magnetic resonance imaging (MRI) revealed a well-defined solid lesion which was revealed intensive enhancement after contrast. On diffusion-weighted images, the lesion showed signiﬁcant diffusion restriction. Three months after therapy, control MRI demonstrated a completely resorbed pineal lesion. The pineal region may be a possible site of metastasis and involvement due to the absence of a blood-brain barrier, and should not be overlooked in patients with not only solid cancers but also ALL.