Purpose To report the clinical features of a rare case of hypotropic dissociated vertical deviation (DVD). Case report A 25-year-old female was referred with unilateral esotropia, hypotropia and slow variable downward drift in her left eye. She had history of esotropia since she had been 3-4 months of age. Best corrected visual acuity was 20/20 in her right eye and 20/40 in the left one when hyperopia was corrected. She underwent bimedial rectus muscle recession of 5.25mm for 45 prism diopters (PDs) of esotropia. She was orthophoric 3 months after surgery and no further operation was planned for correction of the hypotropic DVD. Conclusion This rare case of hypotropic DVD showed only mild amblyopia in her non-fixating eye. The etiology was most probably acquired considering hyperopia as a sign of early onset accommodative esotropia.