Acrokeratosis paraneoplastica (Bazex syndrome) is a rare paraneoplastic dermatosis associated with internal malignancies. Clinical presentation is characterized by erythematous or violaceous scaly plaques involving the digits, nose, ears, palms, and soles. Nail changes commonly present concurrently with cutaneous manifestations. In this review, we characterize nail changes associated with acrokeratosis paraneoplastica. A total of 48 cases were analyzed. Nail findings were nonspecific, with the most common being nail plate thickening, onycholysis, subungual hyperkeratosis, longitudinal ridging, discoloration, and nail plate loss. In most patients, nail changes involved the majority of fingernails and toenails and most often appeared prior to the diagnosis of malignancy. The most common associated underlying malignancies were squamous cell carcinomas of the head and neck. A diagnosis of acrokeratosis paraneoplastica should be considered in patients with onychodystrophy involving multiple nails with accompanying atypical psoriasiform dermatoses. Screening for internal malignancies may significantly decrease morbidity and mortality for these patients.