<b><i>Importance:</i></b> Congenital malalignment of the great toenail (CMGT) is an idiopathic deviation of the nail apparatus. CMGT predisposes patients to recurrent stress forces, microtrauma, and secondary complications. The purpose of this study was to review the current published photographs to determine the relationship between variants of CMGT and the disappearing nail bed (DNB). <b><i>Observations:</i></b> A search in PubMed and Google using the terms congenital malalignment of the great toenail, disappearing nail bed, and lateral nail deviation was performed. Of the 53 photographs found in a total of 35 articles, 23 were disqualified due to low picture resolution or poor angle. The remaining 30 photographs were evaluated. Pure nail malalignment with associated dystrophy and DNB was found in 22 of 30 photographs. Four of 30 cases demonstrated pure deviation of the distal phalanx, with nail dystrophy but minimal DNB. The remaining 4 cases demonstrated a combination of toe deviation and nail unit deviation with varying degrees of DNB. <b><i>Conclusions and Relevance:</i></b> DNB was associated with all forms of pure CMGT. Moreover, a variant of malalignment of the distal phalanx was noted in 8 photographs. This has potential implications for further studies and treatment to correct secondary complications.