Visual memory (VM) declines early in Alzheimer's disease (AD). Besides VM can predict the progression from Mild Cognitive Impairment to AD. However, it is unclear how VM performance relates to markers of AD pathology accumulation—e.g. tau pathology—in individuals who are known to be in the preclinical stage of the disease. In this study we examined the relationship between VM performance and markers of AD pathology in cognitively unimpaired individuals with autosomal dominant AD (ADAD) due to the presenilin-1 (PSEN1) E280A mutation. Forty-six cognitively unimpaired individuals from a Colombian kindred with PSEN1 E280A mutation (20 carriers and 26 age-matched non-carriers) were included. All participants underwent cognitive testing and PET imaging. A VM composite score was computed with the scores of the CERAD constructional praxis recall and the Rey Complex Figure immediate recall. Cortical amyloid accumulation was measured using PiB PET, and regional tau accumulation in the entorhinal cortex (EC), inferior temporal cortex (IT) and precuneus (PCu) was measured using Flortaucipir F18 PET. The mean age of carriers was 35 years, approximately 9 years younger than the expected onset of clinical symptoms. There were no differences between groups in VM performance (p = 0.82). Compared with non-carriers, mutation carriers had elevated levels of cortical amyloid (p < 0.001) and tau (EC: p < 0.001, IT: p = 0.02, PCu: p < 0.01). In mutation carriers, greater amyloid burden was associated with worse VM performance (r = −0.53, p = 0.02). In addition, higher levels of tau accumulation were associated with lower VM performance (EC: r = −0.65, p = 0.002; IT: r = −0.51, p = 0.02; PCu: r = −0.64, p = 0.003). No such relationships were significant in non-carriers. Preliminary findings suggest that subtle changes in VM performance may have a negative association with markers of AD pathology in preclinical ADAD, several years before symptom onset. Further investigation with larger samples is needed to confirm present findings, and to examine the potential usefulness of these cognitive measures for identifying those individuals at high risk to develop dementia later in life.